Physical medicine and rehabilitation is the medical specialty concerned principally with impairments, disabilities, and handicaps that arise following acute or chronic illness. According to the 1980 classification of the World Health Organization (WHO), impairment is physiologic dysfunction or loss of anatomic integrity. Disability refers to functional consequences in relation to self-care and mobility imposed by underlying impairments. Handicap may be defined as a physical condition that interferes with a patient's ability to engage in social, educational, recreational, and vocational pursuits. In essence, handicap compromises patient's full integration into personal relationships and family and societal roles.

Cancer is a group of diseases characterized by uncontrolled growth and spread of abnormal cells. Uncontrolled spread of cancer cells can result in death. Cancer is caused by both external factors (eg, chemicals, radiation, viruses) and internal factors (eg, hormones, immune conditions, inherited mutations). Causal factors may act together or in sequence to initiate or promote carcinogenesis. Ten or more years may pass between carcinogenic exposure or inherited mutations and detectable cancer. Today, cancer is treated with surgery, radiation, chemotherapy, hormones, and immunotherapy.

The National Cancer Institute (NCI) estimates that approximately 8.4 million Americans today have a history of cancer. Some of these individuals can be considered cured, while others still show evidence of cancer and may be undergoing treatment. The 5-year relative survival rate for patients with all types of cancers combined is 59%.

Neoplastic disease can develop in virtually all organ systems in the human body. This unregulated growth injures and compromises organ systems that are functioning normally. Cancer-related diseases often are treated with therapeutic modalities that, in themselves, compromise normally functioning organ systems. Consequently, practitioners of physical medicine and rehabilitation need to respond dynamically, both to disease progression and to various treatments that may contribute to impairment, disability, and handicap.

The rehabilitation approach to treatment of cancer patients originates with the National Cancer Act of 1971. This legislation declared cancer rehabilitation as an objective and directed funds toward development of training programs and research projects. In 1972, the NCI sponsored the National Cancer Rehabilitation Planning Conference. This conference identified the following 4 objectives in rehabilitation of cancer patients:

• Psychosocial support

• Optimization of physical functioning

• Vocational counseling

• Optimization of social functioning

In the 1970s, a number of model systems of cancer rehabilitation programs were initiated and supported through the NCI cancer control program. Cancer rehabilitation can be defined as a process that assists the cancer patient to obtain maximal physical, social, psychological, and vocational functioning within the limits created by the disease and resulting treatment.

Rehabilitation specialists have proposed several general principles regarding rehabilitation interventions for cancer patients. Rehabilitation requires an interdisciplinary team approach because of the variety of potential problems cancer patients may face during the course of illness. Availability of professionals from major disciplines is essential to offering comprehensive care. Patient needs determine the number of team members involved in any given case. The health care team needs to develop rehabilitation goals within limitations imposed by the illness, the patient's environment, and available social support. Goals must be objective, realistic, and attainable within a reasonable time. Structuring goals so that they are attainable within a reasonable time often serves to motivate patients to maintain effort because patients can appreciate gains from active participation in therapy.

Patients, family members, and significant others need to be active participants in the rehabilitation process. Patient and family involvement assists in goal setting. Interdisciplinary rehabilitation is the collaborative effort of professional members of the team working with the patient and accompanying support network. The rehabilitation team needs to provide services to cancer patients throughout the course of illness and during different stages of the disease process. Treatment plans must be individualized to meet each patient's unique and specific needs.

Physicians

The professional clinicians comprising the interdisciplinary team include physicians from several different specialty areas. Primary care physicians, surgeons, radiation oncologists, and medical oncologists make active and concurrent contributions to rehabilitation efforts in an attempt to manage the disease process.

The physiatrist, a specialist in physical medicine and rehabilitation, treats neuromuscular disease, musculoskeletal disease, and functional deficits, in addition to performing electrodiagnostic procedures (eg, nerve conduction studies [NCS], electromyography [EMG]). The physiatrist also prescribes treatments performed by professionals from other disciplines, such as physical, occupational, and speech therapy. The physiatrist serves as liaison between team members. This liaison function requires a considerable degree of coordination, especially when rehabilitation and clinical management of the disease are ongoing at the same time.

Care coordinator (case manager)

The role of the clinical care coordinator is to assist in organization and management of the team. An important aspect of this role is conducting initial evaluations of patients referred to the rehabilitation team for consultation. Care coordinators may originate from nursing, social work, or other rehabilitation-related fields, but they must be familiar with functions of the other disciplines to assess the patient's needs effectively.

Oncology/rehabilitation nurse

The role of the oncology/rehabilitation nurse is pivotal in cancer rehabilitation. The oncology/rehabilitation nurse typically is an extension of other members of the team because he/she frequently must assist with treatment interventions initiated by the physical, occupational, or speech therapists. Such interventions include assisting patients with exercises, mobility on the unit, self-care activities, and speech and swallowing techniques. Because nurses typically have extensive contact with patients and families, they become more aware of the family's emotional stress and adjustment issues. Nurses sometimes function as counselors, providing significant emotional support for patients and their families. In addition to active involvement with representatives of most other disciplines participating in the treatment interventions, nurses are responsible for skin care, bowel and bladder management, and patient/family education. Cancer rehabilitation nurses are crucial in promoting the rehabilitation goal of maintaining optimal independent functioning.

Social worker

The role of the social worker can vary significantly, depending on the medical institution. Social workers often provide counseling services to patients and families regarding emotional support, community resources, finances, lifestyle changes, and treatment participation. In some settings, social workers often serve as leaders for support groups and also may provide active assistance in discharge planning activities, such as arranging home care services and transfer to other health care settings.

Psychologist

Cancer patients and their families often experience a number of psychological and adjustment issues related to illness, treatment, and resulting disabilities. The psychologist provides assessment and treatment to assist in management of cancer-related psychological distress. As a member of the rehabilitation team, the psychologist also assists other team members when psychological issues, either in patients or family members, complicate efforts to provide effective therapy. The goal of consultation of the psychologist with other team members is to maximize the benefit derived by the patient during the rehabilitation process.

Physical therapist

The role of the physical therapist includes evaluation of muscle strength, mobility, and joint range of motion (ROM). Treatment interventions provided by the physical therapist may include therapeutic exercises to maintain/increase ROM, endurance activities, and mobility training (eg, transfers, gait, stair climbing). Physical therapists also can administer various therapeutic modalities, depending on the needs of each individual patient. Examples of modalities that may be beneficial include heat and cold, electrical stimulation, hydrotherapy, traction, and massage.

Occupational therapist

Occupational therapists evaluate patients' ability to carry out tasks related to self-care, including activities of daily living (ADL), such as dressing, bathing, meal preparation, and homemaking. These professionals also assist patients to increase performance of ADL abilities, including use of compensatory techniques and adaptive equipment. In addition, occupational therapists evaluate home environments for potential modification, provide instruction in driving with adaptive devices, and implement interventions to promote upper extremity ROM, strength, endurance, and coordination.

Dietitian

Diet and nutrition are important factors in cancer rehabilitation. A healthy diet and adequate nutrition significantly influence the patient's ability to participate actively in an applied therapy program and are essential for patients undergoing radiation and chemotherapy. The role of the dietitian is to evaluate current nutritional status and provide recommendations regarding specific dietary needs. Patients with cancer often require dietary supplements and alternative foods. Dietitians also assist in teaching patients and family members about the importance of appropriate diet in successful rehabilitation.

Speech therapist

The speech therapist evaluates and treats communication deficits, dysphagia, and cognitive dysfunction in patients with cancer. Speech therapists also train patients in use of alternative means of speech and communication, including adaptive communication devices, laryngeal speech, esophageal speech, and use of a prosthetic larynx. Treatment of patients who have oral defects or experience aphasia also falls within the purview of the speech therapist. Treatment of swallowing deficits that result from illness or treatment also is conducted by the speech therapist.

Vocational counselor

Vocational counselors assist patients in adaptation to the impact of cancer and treatment on employment. Vocational counselors provide evaluation of the patient's suitability for employment and training of the patient, if needed, and serve as liaison between patients and employers. Health care professionals often overlook the impact of cancer on the patient's vocation as an area requiring possible intervention.

Although the professionals mentioned above are the most common members of the cancer rehabilitation team, practitioners from many other fields also provide important and valuable advice, including a chaplain, dentist, orthotist, and prosthetist. Additionally, rehabilitation programs benefit from consultative relationships with other care-providing organizations (eg, home health care agencies, community hospice centers).

Following an initial screening, representatives from other disciplines conduct clinical assessments based on present needs of patient and/or those identified by the care coordinator.

The paradigms of cancer rehabilitation

Dietz identified 4 categories of cancer rehabilitation that address the scope and course of the illness. A variety of approaches to rehabilitation of the patient with cancer includes the following:

Preventative

• Preventative interventions lessen the impact of expected disabilities and emphasize patient education.

• Preventative measures also include approaches to improving the patient's physical functioning and general health status.

• In addition, psychological counseling prior to treatment can assist with early identification of adjustment issues to allow for prompt intervention.

Restorative

• Restorative interventions are procedures that attempt to return patients to previous levels of physical, psychological, social, and vocational functioning.

• Postoperative ROM exercises for mastectomy patients and reconstructive surgery for head and neck cancer patients are representative of this category of interventions.

Supportive

• Supportive rehabilitation is designed to teach patients to accommodate their disabilities and to minimize debilitating changes from ongoing disease.

• Supportive efforts include teaching patients how to use prosthetic devices after amputation, as well as instructing the patient on use of other devices and procedures that assist in self-management, self-care abilities, and independent functioning.

• Other supportive efforts include provision of emotional support associated with adjustment issues while the patient is learning to cope with physical lifestyle changes.

Palliative

• During the palliative phase, when increasing disability and advanced disease process may be present, interventions and goals center around minimizing or eliminating complications and providing comfort and support.

• Palliative goals include pain control, prevention of contractures and pressure sores, prevention of unnecessary deterioration from inactivity, and psychological support for the patient and family members.

J Lehman et al were among the first authors to investigate the frequency of problems that cancer patients encounter in rehabilitation programs. They screened 805 patients with cancer with psychological and physical medicine problems. Patients involved in the study had been diagnosed with a variety of different types of cancers, including leukemia and cancers of the head and neck, breast, respiratory, nervous system, bladder, and bone. Over 50% of patients experienced problems associated with physical medicine, with a significant portion experiencing problems similar to those of other rehabilitation patients.

A large percentage of the sample population demonstrated evidence of psychological problems. Psychological problems were more prevalent in patients experiencing physical problems than those without physical involvement. More than 50% of patients with physical involvement experienced psychological problems, and approximately 29% of patients without physical involvement experienced psychological difficulties. Patients with cancer of the nervous system had higher incidence of psychological problems than individuals with cancer at other sites.

The study concluded that physical medicine and psychological problems coexist in a large number of cancer patients and that many of these patients could benefit from rehabilitation interventions because their problems are similar to problems identified in many other patient populations undergoing rehabilitation.

Patricia Ganz surveyed 500 patients with colorectal, lung, and prostate cancer and found that the average patient had been living with the disease for more than 3 years. She found that more than 80% of this sample reported problems with ambulation and that, for more than 50% of the sample, the problems were severe. In addition, patients with cancer in this sample (41% with colorectal cancer, 69% with lung cancer, and 40% with prostate cancer) reported difficulty with performance of ADL. Physical problems occurred in a relatively functional sample of patients with average Karnofsky performance status scores of 80%. Over 40% of each group had no evidence of active disease. In the sample of colorectal, lung, and prostate cancer patients surviving more than 1 year after cancer diagnosis, Ganz found a wide range of psychosocial problems.

More recently, in a study by VanHarten et al, a questionnaire was devised to address patients' need to receive professional care related to health problems. While 258 cancer patients were invited to participate, only 147 completed the study. The sample consisted of patients with nonmetastatic breast and colon cancer who were living in the community. For all quality of life (QOL) factors, patients could indicate whether they felt need for professional care to contend with cancer-related health problems; 26.5% of patients indicated need for such health care.

Overall, QOL scores were relatively high. Performance of expected roles and mobility were cited as significant problems in 26% of patients. Other patients reported that fatigue and deconditioning interfered with functional performance and mobility. Psychological integration of the new situation into personal relations and coping with daily life also were cited as problematic. As a result of their survey, VanHarten et al proposed a pilot program for patients with cancer in the community.

Components of the program included the following:

1. Fitness and sports activities

2. Relaxation exercises

3. Patient education, especially on disease-related matters

4. Instruction and counseling of patients and relatives on coping strategies, especially dealing with crisis and fear

5. Social and cultural therapy designed to help formulate new and realistic goals in life

6. Dietary advice

A reliable and valid assessment tool is vital for the rehabilitation team to gauge patients' status on admission to the program, during the program, upon discharge from the program, and upon follow-up evaluation. Such an instrument allows each clinician to determine what are reasonable short-term and long-term goals for the patient.

Oncologists were the first practitioners to assess and survey QOL in cancer patients following the advent of chemotherapy treatment. In the late 1940s, Karnofsky and Buchrenal developed a clinical scale to quantify functional performance of cancer patients. Since then, a number of programs intended to ensure QOL have been developed, modified, and used in cancer patients. Key elements in any QOL intervention or in ascertaining overall status of patients in a given clinical situation include the following:

• Physical concerns (eg, symptoms described by the patient)

• Functional ability

• Family well-being

• Emotional well-being

• Spiritual well-being

• Satisfaction with treatment, including financial concerns

• Sexuality and intimacy, including issues of body image

• Social functioning

• Occupational functioning

QOL instruments presently used by clinicians in cancer treatment in rehabilitation setting include the following:

1. Functional living index for cancer

2. European Organization for Research and Treatment of Cancer (EORTC) QOL questionnaire

3. QOL index

4. Cancer rehabilitation evaluation system

5. Functional assessment of cancer therapy

6. Global adjustment-to-illness scale

Acute and chronic morbidity in surgical treatment

Modified radical mastectomy is the most common curative surgical procedure used to treat breast cancer in this country. Breast-conserving surgery is used increasingly for many breast cancers since disease-free survival rates are equal for women who have undergone either procedure. Breast-conserving surgery is associated with improved body image and, perhaps, earlier psychological recovery.

Radical mastectomy removes breast tissue, pectoralis major and minor muscles, and axillary nodes and rarely is used to treat breast cancer today. Modified radical mastectomy removes breast tissue, pectoralis major muscle fascia, and axillary nodes. Total or simple mastectomy removes just breast tissue. Breast-conserving surgery refers to removal of the cancer along with a margin of normal breast tissue and axillary dissection. Wide excision in breast preservation surgery implies removal of a 1-2 cm normal tissue margin, while segmental mastectomy removes even more normal breast tissue.

Quadrantectomy is a procedure to remove the quadrant of the breast that contains the tumor plus the underlying pectoral fascia. Any increase in the extent of surgery is associated with increased risk of both early and late complications. Most reported surgical complications are associated with axillary dissection. Debate still surrounds issues of whether axillary dissection is necessary and, if so, which parameters should be used to determine the extent of axillary dissection.

Principles of wound healing exert direct impact on the initiation of any rehabilitation program and on determination of the appropriate intensity. Wound healing is a dynamic process that lasts months to years. Initially, wounds produce an inflammatory process that lasts a few days unless necrosis, infection, or foreign bodies are present. At the edge of an epithelial wound, basal epithelial cells migrate across the defect on fibrin strands. Epithelial cells cover the wound within 48 hours and thereafter begin to differentiate and keratinize.

Fibroblasts, from the adventitia of blood vessels, migrate into the wound on fibrin strands on day 3 and begin to synthesize collagen fibers, which begin to appear on day 4. Wound strength is related to the rate of collagen formation. By 3 weeks, most wounds achieve 15% of their ultimate strength. Strength increases at a constant rate for 4 months and then at a lower rate thereafter for more than a year. Pain at the wound site generally limits the amount of stress an individual can place on the wound.

Changes in sensation are common, requiring that wounds be treated gingerly. Since external skin sutures may provide a nidus for infection and cause extra scarring, remove them early. Factors that may impede healing include malnutrition (more common in elderly individuals than in younger patients); deficiencies of vitamin A, vitamin C, and zinc; cigarette smoking; and any conditions that decrease tissue oxygenation. Steroid use, radiation therapy, and some chemotherapy agents also impede healing. Administration of Adriamycin, commonly used in adjunct chemotherapy programs, should be delayed until 4 weeks following surgery.

Early complications following mastectomy include seroma formation (10%), wound infection (7%), and skin flap necrosis (5%). Fewer wound infections are seen in patients diagnosed by fine needle aspiration. Immediate reconstruction is not associated with increased rate of complications. Most surgeons agree that a drain must be placed after axillary dissection. Duration of drainage is not based on a standard timeframe, but most surgeons agree that the drain can be removed when the volume of fluid draining from the wound has decreased to less than 20 mL per day. The presence of a drain or seroma can lead to infection. If seroma develops following removal of the drain, most surgeons aspirate the seroma only if the patient is uncomfortable. Do not place a drain in a lumpectomy site since cosmesis diminishes.

Complications associated with axillary dissection are secondary to nerve, vascular, and lymphatic injury. The most common complaints following axillary dissection are reduced sensation under the right arm and decreased ROM of the shoulder. Sensory deficit improves with time but may never return to normal. No known treatment exists for this side effect. Lymphedema can be seen immediately after surgery and results in a small increase in diameter in the upper arm only. Collateral circulation should resolve the edema within several weeks.

Chronic lymphedema and its treatment are discussed later in this article. Injury to the long thoracic nerve results in winging of the scapula. Thirty percent of patients develop serratus anterior muscle palsy secondary to injury to the long thoracic nerve but appear to recover by 6 months. Injury to the thoracodorsal nerve causes slight weakness in internal rotation and abduction of the shoulder from weakness of the latissimus dorsi muscle. Injury of the medial pectoral nerve results in atrophy of the lateral portion of the pectoralis major muscle. Injury to the intercostobrachial nerve results in reduced sensation along the medial aspect of the arm, and, in some patients, subsequent disabling neuralgia develops.

Reconstruction

Intuitively, breast reconstruction offers a woman the opportunity to retain a positive self-image, mitigating concern about breast cancer treatment significantly and perhaps even encouraging women to seek earlier diagnosis of breast cancer. The psychosocial benefit of reconstruction is only slight, however, when patients who have undergone surgical reconstruction are compared to patients treated by mastectomy alone. Body image is affected less by breast-preserving surgery than by mastectomy and breast-reconstructive procedures. Studies report lower scores on measures of body image for women who have undergone breast reconstruction than for patients following breast-preserving surgery. This phenomenon may be related to the complicated nature of reconstructive surgery.

Reconstruction of the breast can be accomplished in several ways at any time following surgery. The type and timing of reconstruction do not affect either biological processes or detection of breast cancer. For advanced cancers where irradiation of the chest wall and regional nodes is planned, breast reconstruction should be delayed, but the intention to perform reconstructive surgery does not prevent radiation therapy if unexpected pathologic findings are discovered.

The simplest reconstruction consists of placing an expandable saline implant under the pectoralis muscle in the musculofascial layer and stretching the tissues of the chest wall to reduce tightness and chest wall firmness, after which the implant is replaced with a permanent implant. Saline is instilled into a fill valve at regular intervals over several weeks until the expander is overfilled to 200 mL beyond the contralateral breast volume. After the chest wall has been stretched to allow for normal breast contour, a second operation is performed to replace the implant with a shaped prosthesis or to remove the excess fluid and fill valve. Complications include extrusion of the expander, infection, and deflation. Patients complain of chest wall tightness and asymmetry.

Flap procedures are used to transfer distant tissue with its own blood supply. Muscle and skin can be transplanted from the back (latissimus dorsi flap), abdomen (transabdominal rectus or TRAM flap), or buttocks (gluteus flap), and a microvasculature anastomosis is performed. The TRAM flap has become the flap of choice because of the volume of tissue that can be moved; however, cigarette smoking, diabetes mellitus, and obesity are relative contraindications because of decreased microcirculation. When reconstructing the irradiated chest wall, the TRAM flap is preferred because of its vascularization. Generally, a TRAM flap procedure requires that the patient be hospitalized for 5-7 days and that 2-3 months be allocated for recovery. Loss of the rectus muscle can result in abdominal wall hernia (2-5%) and postural changes.

If a TRAM flap reconstruction is planned, address rehabilitation issues and counsel the patient preoperatively about the need for a program to address back and shoulder strengthening. Decreased trunk flexion and extension strength also result from the surgery. Physical therapy focuses on strengthening exercises and compensatory movements for most patients, particularly for individuals with chronic spine pain.

Other types of reconstruction are associated with discomfort related both to loss of tissue from their respective areas and to the actual surgical procedure. The latissimus dorsi flap procedure is a less complicated operation than other reconstructive procedures, but an implant is required for adequate cosmesis. The most common complication is seroma formation. No functional loss of shoulder strength is seen. A gluteus maximus flap is both less painful and less morbid than a TRAM flap, but it is more technically demanding. A nipple can be constructed in all types of reconstruction by puckering skin and tattooing an areola, or by grafting skin into a nipple site and tattooing. Avoid grafts on irradiated skin.

Shoulder and arm rehabilitation

The goal of arm and shoulder exercises is to enable the patient to return to normal activity after axillary dissection. At 3 or 15 months after surgery, approximately 80% of patients continue to report at least 1 problem. Problems may include swelling (25%), weakness (25%), limited ROM (30%), stiffness (40%), pain (50%), and/or numbness (55%). Increasing numbers of complaints are associated with higher levels of psychological distress. Optimally, evaluate the patient preoperatively for strength, ROM, sensation, posture, endurance, and general functional ability. Provide the patient with instructions regarding ROM exercises, postoperative breathing, and initial mobility following surgery. Start shoulder and arm rehabilitation as soon as the surgical incision appears healed and recurrent seroma or infection is absent, remembering the principles of wound healing.

Early physical therapy to the shoulder following axillary dissection does not increase incidence of lymphedema. Development of seromas is more prevalent in more extensive surgeries. Encourage patient to begin gradual stretching exercises for all degrees of motion within a few days of surgery. The optimal program starts postoperatively with gentle ROM exercises of the shoulder from 45-90° in patients without reconstruction. PROM should start to 90° of flexion and abduction with external and internal rotation as tolerated. Early mobilization of the glenohumeral joint improves shoulder ROM. Faster recovery was found in patients who began shoulder flexion to 40° on day 1 and 90° on day 4 compared to delayed initiation of ROM exercises. Methods to compensate for nerve injury both improve muscle strength and avoid shoulder tightness and discomfort.

Patients should begin full shoulder and arm ROM exercises as soon as the surgeon deems them safe, often after removal of the drain(s). Active and active-assistive exercises can be increased at this stage. Exercises such as wall climbing, pulley, and wand should be added. After removal of all sutures, more aggressive exercises can be incorporated.

Physical modalities may be helpful. Use ultrasound with caution, given potential risks of promoting residual tumor cell growth or metastasis. Include stretching exercises and electrical stimulation as part of the rehabilitation program. Patients treated by mastectomy, rather than breast-conserving surgery, are more likely to experience impaired mobility. Prospective studies demonstrate that patients who receive structured physical therapy achieve better arm and shoulder function than those who do not.

A home exercise program should be given, and follow-up physical therapy assessment should be included. Scar massaging usually is incorporated in this program around 1 month postoperatively. With radiation treatment, ongoing ROM exercises are particularly important to avoid contracture formation.

Discuss lymphedema precautions with the patient preoperatively and review her condition within several days of surgery. When resting, the patient should elevate her arm higher than her heart, but not over her head. Exercises using the forearm and hand should be instituted immediately to help muscular propulsion of blood and lymph fluid from the lower arm. Encourage the patient to squeeze a tennis ball or other soft ball when resting. Advise the patient not to lie on her arm in the ipsilateral decubitus position and to avoid a prone position.

Discuss the effects of skin or soft tissue infections on development of arm edema, the effect of gravity on lymph drainage, the importance of avoidance of procedures on the arm that may break the skin, and the type of exercises that can improve muscle tone in the arm. Encourage the patient to be conscious of the importance of weight management since development of edema of the arm is associated with weight gain. Advise patient to call her physician immediately if signs of erythema or swelling occur. Many physicians prescribe antibiotics for acute development of edema.

Consequences and treatment of radiation therapy

Use of radiation therapy after breast-preserving surgery is common to reduce the probability of recurrence within the breast and after mastectomy when risk of recurrence within the chest wall is high. The breast is treated with tangential techniques that also include irradiation of the underlying muscle, rib, and the anterior surface of the lung. After mastectomy, the chest wall is treated with similar techniques, but radiation is delivered after subcutaneous tissue has been damaged by production of skin flaps. The supraclavicular, axillary, and sometimes the internal mammary nodes are irradiated when risk of nodal recurrence is high. Direct anterior fields are used, treating greater volumes of rib and lung tissue. The brachial plexus often is within the node fields, but damage is uncommon with standard doses. Axillary node radiation is associated with higher risk of lymphedema; avoid it unless significant risk of axillary node recurrence exists.

The effects of surgery are exaggerated by radiation. Fibrosis secondary to radiation in the treatment field may cause the following:

• Increased obstruction of arm lymphatics (if within the radiation field)

• Increased tightness of the chest wall and pectoralis causing decreased shoulder mobility (more prevalent in mastectomy patients)

• Pain in subcutaneous tissues, intercostal muscles, or ribs

• Decreased pulmonary reserve (rare unless more than 10% of lung volume is treated)

• Rib fractures (1% risk)

Soft tissue infections, cigarette smoking, and diseases that may impair microcirculation (eg, diabetes, arteriosclerotic vessel disease) increase the probability of fibrosis. Exercise and manual massage may decrease pain and discomfort associated with fibrosis. Dry skin ointments may comfort dryness and itching. Breast edema is a side effect unique to breast preservation and is related to the extent of axillary dissection, location and extent of breast surgery, and breast size. Weight gain may aggravate breast edema. Breast edema resolves with time, but weight loss, proper breast support, and avoidance of prone sleeping position may help. Diuretics rarely are helpful. Benzopyrones currently are not available in the US. Development of late breast edema is uncommon and may represent infection or recurrent cancer.

If large volumes of lung tissue (greater than 10%) are included within radiation fields, the patient may develop cough, shortness of breath, and low-grade fever 4-12 weeks following radiation. The physician must rule out an infectious source. Chemotherapy increases the risk of pneumonitis. Temporary low-dose steroids may relieve symptoms of radiation pneumonitis, and often antibiotics are added empirically. Acute radiation pneumonitis resolves in 2-3 months and does not predict long-term pulmonary insufficiency. Ten percent of lung volume must be treated to observe pneumonitis. Always compare chest x-rays with radiation portal films to assure etiology of the disease process.

Most patients suffer subclinical effects on the lung. Diffusing capacity of carbon monoxide drops in most patients but returns to normal levels by 24 months; however, cigarette smokers demonstrate greater deficit and less recovery. Cigarette smoking affects lung tolerance to radiation, so encourage patients to stop smoking. Permanent injury to the lung because of interstitial fibrosis is localized to the radiation field only and can be identified on lung x-rays; long-term effects of lung fibrosis are related to volume of irradiated lung and the patient's pulmonary status prior to radiation.

Radiation-induced brachial plexopathy is characterized by shoulder discomfort and progressive paresthesias and weakness in the arm and hand. About 1% of patients who receive nodal irradiation with doses greater than 50Gy and who usually are treated with chemotherapy develop problems. If doses are limited to 50Gy, symptoms generally are transient. Symptoms develop 3-14 months after radiation and commonly affect distribution of the lower plexus. Progressive neurologic dysfunction of the brachial plexus is associated with radiation fibrosis because of large fraction sizes. Increasing prevalence of pain in addition to paresthesias of the hand and proximal arm weakness may be observed. Weakness in distribution of the upper plexus is more common. Associated arm edema secondary to radiation often is noted. No known treatment exists, other than symptomatic management; however, cancer infiltration of the brachial plexus can mimic these symptoms and must be ruled out.

Women treated with direct fields to the left chest may have increased incidence of arteriosclerotic heart disease and, consequently, of myocardial infarctions. Women often are made menopausal by estrogen deprivation, which may add to incidence of cardiovascular disease. Discuss benefits of diet, exercise, hypertension treatment, and treatment of cholesterolemia with any patient with breast cancer, but the significance is more obvious in patients treated with radiation and chemotherapy.

Hormonal treatment

Tamoxifen commonly is prescribed for women with hormone receptors positive for estrogen whose cancers are more than 1 cm in size. Many premenopausal women receive tamoxifen after chemotherapy, while many postmenopausal women with large tumors or positive nodes receive it as single-agent adjunct therapy. Tamoxifen is prescribed for a minimum of 5 years. In addition to the antitumor effect, other benefits of tamoxifen include reduced bone loss and improved lipid profile. Tamoxifen often exaggerates symptoms of estrogen deprivation, with hot flashes (50%- 60%), depression (10%), weight gain, and vaginal dryness as common complaints. Examine patients annually for possible risk of endometrial carcinoma secondary to tamoxifen.

Chemotherapy consequences

In the adjunct setting, chemotherapy usually is administered in 4-6 cycles of 3-4 weeks. Preconceived notions, often incorrect, impact a woman's attitude toward chemotherapy. Anticipating these concerns, particularly nausea, hair loss, and lifestyle changes, when introducing the topic of chemotherapy, is important. Immediate effects of chemotherapy include general fatigue and nausea and vomiting. The latter can be countered effectively with medication, including prochlorperazine, lorazepam, ondansetron, and granisetron. Patients often gain weight since food may relieve nausea, and the basic metabolic rate also may drop. Fatigue can be overwhelming and can affect exercise and activity levels. Work and family issues may be important during chemotherapy since treatment can last for many months.

Diminished immune status occurs for many women during chemotherapy, putting them at risk for infection. These periods are short, but some women require either increased breaks between chemotherapy cycles or use of growth factors, which are associated with their own side effects. Prolongation of chemotherapy may be devastating for many women who have planned for disability periods of a certain length and are limited in sick absences from work or must rely on childcare. Generally, these women should avoid being around children with the usual childhood diseases (eg, chickenpox).

Chemotherapy may render women, generally those in their late 30s or 40s, menopausal. Incidence of premature ovarian failure is about 70%, but it is less common in women aged less than 30 years. The most common severe late effect of Adriamycin chemotherapy is cardiomyopathy, occurring in fewer than 1% of women with a total cumulative dose of 300 mg/m². A previously active young woman may find herself dyspneic on exertion. Appropriate cardiology consultations and cardiac rehabilitation programs may improve performance status of women made symptomatic by therapy. Another serious side effect of chemotherapy is increased risk of leukemia, which is related to dose and type of alkylating agent (incidence 0.7% at 10 years) and may increase with adjunct radiation. Current data suggest that leukemia risk is minimal with regimens containing cyclophosphamide used today.

Encourage women to be active and to seek support. Evidence suggests that support groups or a confidant increase probability of survival. Continuation of regular activities during chemotherapy is beneficial. In one study, 41% of women found that treatment was easier than expected. By focusing on delayed benefits of chemotherapy (ie, survival issues), women can cope with short-term adverse psychological effects. In some professions, women are not allowed to continue working during therapy (eg, firefighter, airline pilot), and they are placed on medical disability. The Americans with Disabilities Act (ADA) protects women with breast cancer from workplace discrimination in most settings. The Family Medical Leave Act (FMLA) also requires flexibility in scheduling for patients and family members to accommodate treatments.

Exercise

No controversy surrounds the issue of beneficial effects of exercise for the general population on the cardiovascular system and weight. Women with breast cancer who participated in aerobic exercise demonstrated improved QOL. Obesity is a minor risk factor for development of breast cancer and is associated with more complications from breast cancer treatment (eg, lymphedema), as well as with higher risk of breast cancer recurrences. Exercise produces improvement in functional capacity of breast cancer patients on adjunct chemotherapy. Weight gain is common during chemotherapy, apparently connected with loss in muscle tissue, which may contribute to reduced functional capacity and lower metabolic rate during the period of adjunct chemotherapy. Increase in lean body weight is observed in patients who exercise while on chemotherapy.In animal models, not only was exercise shown not to induce metastases, but also fewer metastases were seen in exercise-trained animals. Exercise also has been shown to attenuate cachexia in animals.

Lymphedema

Any dissection of axillary lymphatics and nodes places a woman at risk for edema of the arm. Axillary surgery and radiation can lead to lymphedema, which may be caused by direct damage to axillary lymphatics. Fibrosis of the axilla secondary to surgery and/or radiation causes venous and lymphatic obstruction by compressing major vascular trunks and blocking regeneration of lymphatic and venous collaterals. Additional radiation therapy, trauma, and infection are other causative factors. Increase in arm circumference immediately after surgery is common and should resolve within weeks. No standardization exists in the literature as to the type and location of measurement and the implications of such measurement. Most clinicians agree that a difference in circumference of more than 2 cm between the arms has clinical significance.

Development of late arm edema is associated with age, extensive cancer within the axilla, extent of axillary dissection, and dose and techniques for administration of radiation. Nearly 33% of patients aged greater than 55 years and 25% of patients with more than 15 nodes dissected developed 2 cm or greater difference in the circumference of their arms by 3 years. Late breast edema, after axillary dissection performed in conjunction with breast-preservation surgery, is less common, so always consider the presence of an infection or recurrent cancer as possible causes for late edema.

Perform medical assessment to determine the cause of swelling. Rule out or treat infection, venous thrombosis, or cancer recurrence. Prescribe antibiotics if development of edema is acute. Make serial measurements of both arms with the olecranon as the reference point. Assess shoulder, arm, and hand strength; sensory changes; color; turgor; pulses; and mobility. In rare cases, long-standing lymphedema can lead to lymphangiosarcoma, a highly aggressive tumor with poor survival despite forequarter amputation.

Conservative management of lymphedema should include preventive and mechanical modalities as needed. Pharmacologic means include antibiotic prophylaxis to prevent and treat cellulitis and lymphangitis. Drugs such as anticoagulants, hyaluronidase, pyridoxine, benzopyrones and others have been used but have no proven therapeutic value. Preventive care should emphasize identification of patients at highest risk of lymphedema. Comorbid illnesses such as hypertension, heart disease, diabetes and kidney disease can contribute to edema also. Patients should understand lymphatic drainage, pathology leading to lymphedema, signs, symptoms and complications of lymphedema. Self-care instructions include the following:

• 1) Proper nutrition with balanced nutrition, higher protein, and lower salt intake

• 2) Weight management

• 3) When possible, the arm should be elevated above the level of heart.

• 4) Home exercise program
  • ROM exercises
  • Exercises and techniques to improve venous drainage
    
  • The importance of gravitational drainage
    
  • Static resistance exercises and positional changes need to be incorporated into daily activities, including positioning for sleep.
    
  • No heavy lifting with the involved arm, typically suggest less than 15 lbs

• 5) Injury and infection should be avoided.
  • No venipuncture or finger sticks on the involved side
  • Skin breaks should be cleaned with mild soap and water, followed by antibacterial ointment use.
    
  • Recommend long-sleeved shirts and bug-repellents for prevention of bug bites.
    
  • Use of gloves during gardening
    
  • Use of electric razor for shaving
    
  • Good nail care, including not cutting the cuticles
    
  • Gauze wrapping instead of tape use
    
  • Physician should be notified about rashes, erythema, swelling, pain, increased warmth or localized infection.

• 6) Daily cleaning and lubrication of skin

• 7) Avoid constrictive pressure on the arm.
  • No blood pressure cuff
  • No constrictive bands

• 8) Recommend follow up with the physician regularly and with sudden change in arm circumference, as well as with evidence of infection.

Cancer syndromes, either as a consequence of tumor-induced organ system injury or of toxic therapeutic interventions, can produce inactivity in the patient. Fatigue and, in more advanced conditions, asthenia, cachexia, and anorexia compound underlying organ system injuries. Effects of inactivity contribute to morbidity and mortality by predisposing organ systems to further pathophysiological risks. Various deleterious effects of inactivity have been documented in both healthy individuals and patients with cancer.

Musculoskeletal

Strength declines in healthy individuals placed on complete bedrest at a rate of 1-1.5% per day, or about 10% per week. Muscle torque may decline as much as 24% in lower extremity muscles after 5 weeks of bedrest. Loss of proximal lower extremity strength often is greater than that seen in the upper extremities, which leads to impairments in assuming a sitting and standing posture and in ambulation. Muscle shortening occurs in addition to loss of muscle force. Muscle shortening, in conjunction with changes in periarticular and intra-articular tissues, contributes to production of joint contractures. If local edema and hemorrhage are present, collagen formation escalates, producing soft tissue tightness. In the presence of underlying muscle weakness, as might be seen with lower or upper motor neuron lesion, decreased levels of activity add to weakness already present. Dynamic muscle imbalance in these settings also further increases the risk of joint contracture.

Urinary calcium excretion increases within 2-3 days of bedrest and continues to increase over 4-7 weeks. This hypercalciuria may result from loss of muscle pull on bony surfaces and eventually leads to disuse osteoporosis. In younger individuals, shift of calcium from bone to the circulatory system is heightened and exceeds maximal urinary excretion, sometimes resulting in hypercalcemia. Underlying skeletal metastatic disease or paraneoplastic production of compounds similar to parathyroid hormone (PTH) may place these patients at risk for hypercalcemia. In one study of subjects on bedrest, 8 hours of sitting and 4 hours of supine exercise per day were insufficient to diminish hypercalciuria, while standing 3 hours per day was helpful.

Respiratory

When a person assumes a recumbent position, the diaphragm moves cephalad because of pressure from intra-abdominal contents, effectively decreasing intrathoracic size. Though lying down initially causes an increase in pulmonary blood flow as blood redistributes from the lower extremities, within 60-90 minutes pulmonary blood flow drops back to baseline or below the level seen while the patient is sitting. Abdominal muscle activity predominates over rib cage motion when the patient is lying down, producing a shallow breathing pattern and increased respiratory rate. Reduction in diaphragmatic and intercostal muscle activity contributes to weakness of the respiratory musculature, just as inactivity causes weakness in the extremity musculature.

Reduction in rib cage motion can lead to tightness of the costovertebral and costochondral joints. As a result of these anatomic changes, functional residual capacity declines, and closing volume (the point during expirationwhere the alveoli close), which is not affected by changes in position, may increase beyond functional residual capacity, producing atelectasis. Coughing to clear secretions is more difficult in the supine position, so pooling of secretions occurs more in the dependent portions of the lungs. Conversely, blood flow is greater to these same lung portions, leading to a ventilation-perfusion (V/O) mismatch, producing arterial hypoxemia.

Several factors put the patient with cancer at higher risk for respiratory complications than the general population. Coughing or taking deep breaths may be painful for the patient with rib metastases or for the patient who has undergone surgical procedures of the chest and abdomen. Lung involvement, because of primary tumor, metastatic disease, malignant pleural effusion, or complications of chemotherapy or radiation, further contributes to reduced oxygenation, retained secretions, and risk of pneumonia. Frequent position changes may improve secretion clearance and V/O mismatch in patients on bedrest. Encourage patients to take deep breaths at regular intervals and to use incentive spirometers and pulmonary resistive exercises. Stretching and strengthening of the trunk and abdominal muscles can help to prevent or treat rib cage tightness and weakness.

The urinary tract

Voiding in a supine position inhibits effective bladder evacuation. Stasis of urine occurs within the renal pelvis, and this urinary stasis, in conjunction with the hypercalciuria associated with immobilization, predisposes a patient to development of stones within the urinary tract. Retention of urine or use of indwelling catheter increases risk of urinary tract infections. Patients with cancer involving bladder outlet obstruction, as in prostate cancer, or with impaired bladder emptying caused by involvement of sacral nerves or spinal cord, are at added risk when required to void on a bedpan.

Prevention of urinary tract complications involves limiting use of indwelling catheters as much as possible; if long-term catheter use is required, consider a condom catheter in the male or intermittent catheterization in the female. Provide a bedside commode for patients with intact spontaneous voiding to allow them to void in a more upright position when they can be transferred. Allow patients bathroom privileges as soon as they are able to move about.

The gastrointestinal system

Inactivity results in impaired colonic function. Immobilized subjects show an increase in adrenergic stimulation, resulting in decreased peristalsis and increased sphincter contraction. Studies using radiopaque markers demonstrate an increase in colonic transit time and decline in mass propulsive waves of the colon in immobilized individuals. Constipation may occur when the patient is receiving opioids for pain control and may result in fecal impaction. Administration of chemotherapy may result in nausea, vomiting, and anorexia. These factors, in combination with the negative nitrogen balance associated with bedrest, may further contribute to cachexia and hypoproteinemia. Early encouragement of patients to use the bathroom or commode and practice of a consistent bowel program, including use of stool softeners and bulk-forming agents, can reduce risks of constipation.

The cardiovascular system

Hemodynamic changes associated with compromise within the cardiovascular system begin within a few days of recumbency. Healthy young men lose 300-500 mL of plasma volume within the first week of bedrest. Plasma volume declines more than red cell mass, producing an increase in blood viscosity, which is thought to contribute to the risk of deep vein thrombosis (DVT). Hypotension in connection with upright positioning has been found in patients within a week of beginning a regimen of bedrest. When healthy individuals are elevated to an upright position, venous return declines, decreasing stroke volume and cardiac output. Normally, adrenergic sympathetic stimulation occurs, producing increase in the heart rate and vasoconstriction of peripheral and splanchnic blood vessels, maintaining blood pressure.

Following a prolonged period of recumbency, the circulatory system is unable to produce adequate vasoconstrictive response to changes in posture, leading to fall in blood pressure and tachycardia when the patientrises to a standing position. Stroke volume and cardiac output decline, producing lightheadedness and syncope secondary to inadequate cerebral perfusion. Additional symptoms (eg, burning in the lower extremities, nausea, diaphoresis) also have been documented after recumbency, although significant drops in blood pressure may not be found in all cases when the patient is assuming a standing position. Decreased cardiac efficiency also is affected in response to exercise. Increases in stroke volume in response to exercise are not maintained, and cardiac output declines. In patients with coexisting coronary artery disease, changes upon standing may precipitate myocardial ischemia. Maximal oxygen consumption decreases by as much as 15% when healthy individuals exercise in an upright position after 10 days of bedrest. Once this postural response is lost, 3-4 weeks may be required to establish normal postural responses.

Bedrest, in association with other risk factors, may result in development of DVT, and risk for thrombosis increases with the length of bedrest. In addition to changes in blood viscosity, mechanical compression of veins may contribute to venous stasis. Cancer patients, because of associated hypercoagulable states, are predisposed to formation of venous clots. Several strategies can be helpful in prevention and remediation of cardiovascular complications, although early mobilization of the patient is the most effective approach. Maintenance of adequate fluid and salt intake is another simple measure for alleviation of symptoms associated with cardiovascular symptoms.

Dynamic resistance exercises in supine position assist with maintaining plasma volume. Abdominal strengthening and lower extremity exercises (eg, ankle pumps) improve venous stasis and can be performed in conjunction with use of elastic stockings and abdominal binders to maintain blood pressure in orthostatic patients. Use of reclining wheelchairs or tilt tables may help the patient adjust gradually to an upright posture if orthostatic symptoms are a problem. Introduce pharmacotherapy in cases of suspected autonomic neuropathy-related orthostasis. Recommend that the patient begin sitting upright as soon as possible because lack of orthostatic stress significantly contributes to impairment in exercise capacity.

The nervous system

Balance and coordination decline in patients on bedrest. This decline may increase a patient's risk of falling. Confinement of a patient to a hospital bed also can cause sensory deprivation, which has been found to affect perception and cognition with documented changes in concentration, sensory distortion, and hallucinations, both in healthy subjects or hospitalized patients. Alterations in intellectual and perceptual testing also have been found in patients on bedrest. Early activity with access to sensory stimulation can be helpful in prevention of changes in intellectual or perceptual capacities.

The skin

Hospitalized patients are at high risk for development of pressure ulcers, with 7.7% incidence within 3 weeks in high-risk patients. Geriatric patients are at particular risk for pressure ulcers because of loss of subcutaneous tissue, decreased connective tissue elasticity, and decreased secretion levels of sebaceous and sweat glands associated with aging. Sustained pressure over bony prominences results in ischemic injury, and, because muscle and subcutaneous tissues are more sensitive to injury than the epidermis, initial appearance of a sore may not reflect the severity of the underlying injury. Several factors contribute to skin breakdown, including pressure, shearing forces, friction, and moisture.

Upper extremity

Upper extremity wide excisions and limb salvage have not caused great problems within the rehabilitation process, especially in terms of pain, edema, or limitation of strength or motion. Instruct the patient how to gain maximum use of the extremity. Individuals who have had nerve resections and limb-preserving procedures need the most input.

Tumors found in the upper extremity generally are smaller than those found in the lower extremity, allowing for more conservative or limited surgery. Whole muscle groups are removed only rarely. If any muscles or nerves are resected close to the wrist or hand, the hand may become insensate. In this case, the procedure of choice would be amputation.

Postoperatively, morbidity in the upper extremity is less than in the lower. Suction drainage is less prolonged, wound infection is less frequent, and radiation is better tolerated most of the time. The anatomic location of the tumor and volume to be treated determine the reaction.

The interdisciplinary services under the direction of physical medicine and rehabilitation specialists have much to offer patients undergoing Tikhoff-Linberg procedure. These patients are likely to retain hand function and some elbow function, but lose shoulder function. Outcome clearly is superior to that in forequarter or shoulder disarticulation procedures. Further, Tikhoff-Linberg procedure is minimally disfiguring and is associated with only mild-to-moderate pain and edema. The patient's acceptance of the procedure and its outcome generally has been good.

The rehabilitation process begins with a patient orientation program. Often, the patient views pictures of other patients who have undergone the same procedure, demonstrating what they can do postoperatively and what limitations in function are likely. Next, a shoulder mold is fashioned, using the involved shoulder, provided that its contours are not distorted. Heat-moldable material is used. The cosmetic shoulder helps preserve the symmetry and appearance of the shoulder contour and can support a bra strap or heavy overcoat. This cosmesis is the same as that provided following forequarter amputation. In patients in whom the deformity following surgery is minimal, a commercially available shoulder pad may suffice. Use of these devices is optional.

Clothing options for women include purchasing blouses with asymmetric, or off-center, closures and using decorative scarves to mask the body contour. On the first postoperative day, an arm sling is provided for support and to restrict abduction. Maintain motion restriction until the incision is healed (usually about 2 weeks). Control edema, when present, with an elasticized glove or elastic stockinette. At the same time, recommend active maximal hand movement to preserve strength and ROM and to help mobilize edema through pumping action of muscles.

Teaching the patient to be aware of proper head and neck positioning and cervical ROM is initiated during the first postoperative days (or when patient first becomes ambulatory). When permission is given to begin motion, usually at 2 weeks postoperatively, recommend active and active-assistive elbow motion within the confines of the sling. At about 3 weeks, remove the sling for passive shoulder ROM and wrist pronation and supination. Discontinue use of the sling after the suture line is healed, but recommend its use for upright activities in which arm support increases comfort.

Joint immobilization for less than 2 weeks results in capsular adhesions that are overcome easily. Longer periods of immobilization often result in fixed contracture; advise patient to avoid immobilization. Once the arm is out of the sling, recommend performing full elbow and wrist ROM (eg, flexion, extension, pronation, supination) for several minutes daily. Advise the patient to perform passive shoulder ROM (eg, flexion, abduction, external and internal rotation) and pendulum exercises for several minutes daily with the help of a family member or health professional. Recommend use of bathroom equipment (eg, grab bars, tub seats) to enhance safety for these patients. Encourage the patient to resume his/her normal daily activities. Advise the patient not to lift more than 20 lb with the arm that has undergone the Tikhoff-Linberg procedure. Modified tennis and even rowing activities can be performed following rehabilitation. Pain and shoulder or arm dysfunctions are not significant management problems. Painoften is controlled with modest analgesia.

Partial or total scapulectomies are procedures performed when tumors involve the scapula or surrounding soft tissue. Removal of all or part of the scapula, including the glenoid fossa, may be necessary. If the glenoid complex is left intact, upper extremity function may be close to normal. Removal of the glenoid creates restrictions of arm movement, often actively beyond 90°. Pain and complaints of fatigue at the end of the day are not uncommon. A sling for temporary support may be adequate because dependency increases chances of edema.

The deltoid muscle mass forms the roundness of the shoulder and moves the upper extremity at the glenohumeral joint. Following partial resection, the arm usually is held in a sling until drainage subsides. Do not initiate active ROM at the shoulder until sutures have been removed although external rotation can be started with the arm held at the side. Patient can perform full elbow motions. At the time staples or sutures are removed, have the patient perform active and resistive exercises. No chronic residual problems have been observed in patients with partial deltoid resections.

Vital structures adjacent to soft tissue sarcomas of the axilla often are difficult to define. Proximity to the brachial plexus may be impossible to discern unless the patient has neurologic signs. Proximity of the tumor to the humerus is difficult to identify, despite sophisticated scanning devices. Adjacent musculature may need to be sacrificed, such as the long head of the triceps from its origin or the latissimus dorsi as it approaches the axilla posteriorly. Generally, if deeper structures are involved, surgery to remove the sarcoma cannot be performed except by forequarter amputation or Tikhoff-Linberg procedure.

After sarcoma excision in the axilla, keep the arm in a sling until drainage subsides, possibly for more than 2 weeks since this area is associated intimately with major lymphatic channels. If radiation is prescribed, position the patient's shoulder at least 100° abduction/flexion and 75° external rotation, probably one of the most difficult postures to assume without discomfort following surgery. Suggest use of electrical stimulation to decrease pectoral muscle spasm, a great inhibitor to full shoulder ROM and other modalities. Have the patient assume this position when radiation is being given to minimize radiation exposure to the breast and upper arm. Physical therapy treatment may be needed twice a day. Once the shoulder can be moved about 90°, generally no problems are encountered until the area becomes sensitive to radiation.

Skin breakdown is not uncommon and delays delivery of radiation treatment. Suggest that the patient wear 100% cotton T-shirts for absorbency. No deodorants or body creams are allowed unless recommended by the radiation therapist. Recovery of arm motion becomes easier the second and third time radiation therapy is resumed, but, throughout the course of treatment, the program must be repeated. Chronic lymphedema is common. An elastic stockinette or a customized sleeve may be adequate to control swelling. If lymphedema is severe, use of an intermittent compression machine is recommended.

If the brachioradialis muscle must be excised, the elbow should be protected in a splint until closed suction drainage slows and healing is underway. Once this has occurred, proceed with active ROM to the elbow as tolerated. If radiation must be applied to the antecubital fossa, the tendons of the biceps and brachialis muscles may become fixed. The brachial artery and the median nerve may become enclosed in scar tissue. Damage to any or all of these structures can cause secondary problems, such as an insensate nonfunctioning hand, at the very worst, or a weak elbow. With soft tissue sarcoma adjacent to the head of the radius and radial nerve, the elbow is vulnerable over the surgical area. Consider having a protective device made from thermoplastic material, or provide a commercial elbow protector. In rehabilitation, emphasis is on maintaining a functional position during elbow and finger ROM.

Fabricate a dynamic splint with wrist and fingers stabilized in functional position so that finger flexors and interossei can function well in grasping. After complete recovery from surgery and radiation, attempt tendon transfers using the flexor carpi radialis and thumb stabilizers.

Trunk

Retroperitoneal tumors are difficult to excise and often recur because of problems in attaining negative surgical margins. Physical therapy usually is requested in conjunction with adjunct radiation therapy. The femoral nerve frequently is within the radiation field, resulting in the need to protect and support the quadriceps muscles. Edema is a secondary complication if the inguinal nodes lie within the field. Recommend use of support stockings along with elevation of the lower extremities throughout the day.

A buttockectomy is performed when there is en bloc resection of the gluteus maximus muscle. The surgeon must be careful not to damage the sciatic nerve intraoperatively. Closure of the incision may be tenuous if large amounts of skin are removed. The patient may complain of difficulty climbing stairs, pain along the incision, and altered body image. Radiation that includes the buttock disrupts normal sexual functioning and bowel habits. The physical therapist should encourage strengthening of other hip girdle muscles and provide seat cushions. A custom buttock cosmesis may be fabricated out of thermoplastic material to resemble the contralateral buttock. The buttock cosmesis is secured to the undergarments with Velcro. Seat cushions or wedges may be needed for the patient to sit comfortably and provide symmetric weight bearing on the buttocks.

An internal hemipelvectomy may be indicated with diagnosis of soft tissue sarcoma in the upper thigh and/or buttock or low-grade sarcoma of the pelvic bones. The sacrum is transected through the neural foramina with resection of the hemipelvis, proximal femur, and, occasionally, bladder, rectum, or genitalia. In cases of an intrapelvic tumor, entering the peritoneal cavity is inevitable in surgery. Stabilization of the pelvis and femur requires prolonged bed rest with skeletal traction to allow for fusion and maintenance of as much leg length as possible.

Recommend shoe lifts as soon as bed restrictions are discontinued, usually between 3-6 weeks postoperatively. Partial weight bearing is allowed on crutches until a fibrous union of the remaining pelvis or ilium occurs with the femur, which may take up to 6 months. Emphasize the importance of strengthening the distal muscles and upper extremities through repetitive active exercise against gravity. Sensation generally remains intact, and few patients complain of pain. Variations of this procedure are common, and the therapist and surgeon/physiatrist should maintain a close relationship to monitor progress. At 6 months, the patient can walk on all surfaces with only a cane and/or shoe lift to equalize pelvic height in cases of leg-length discrepancy.

If the sciatic nerve is sacrificed, motor loss is inevitable, combined with leg anesthesia and tendency for skin to ulcerate with trauma. Recommend an ankle-foot orthosis (AFO) to assist with foot clearance. Following initial treatments, suggest ankle fusion or a posterior tibialis transfer procedure. Educate patients on proper foot care, choice of shoes, and orthotic application.

Lower extremity

The thigh is one of the most difficult anatomic areas in which to attain local tumor control without significant morbidity; it is, historically, an area most likely to develop soft tissue sarcoma. Tumors discovered in the lower extremity generally are large because they have been masked by bulky muscle tissue. Morbidity involved in irradiating the upper medial thigh and groin potentially is severe. Because of radiation scatter, sexual dysfunction is probable. Chronic lymphedema following irradiation of the lymph node complex in the groin frequently is observed. Hip joint dysfunction and pain are not usually symptoms but may be late findings.

For most wide local excisions of the thigh, serosanguinous drainage is prolonged. When drainage has decreased or suction tubing has been removed, initiate ambulation and active exercises in earnest. Suggest use of commercial immobilizers to protect the lower extremity from poor positioning, but also to prevent wounds from being inadvertently overstretched, particularly when incisions cross a joint.

A large soft tissue sarcoma within the anterior thigh group may require excision from origin to insertion of the whole quadriceps muscle. This procedure is reserved for high-grade tumors. Lower tumor grades may be excised adequately by removal of some portion of the quadriceps.

Radiation treatment usually is not required with formal muscle group excision because radiotherapy would include 2 joint spaces and would be a most morbid procedure. If the patellar tendon were irradiated with 6,000 rads or more, tendon breakdown would occur over time.

At approximately 2 weeks postoperatively, a dual channel metallic AFO is provided to block out dorsiflexion and allow only 5° of plantar flexion. Encourage use of a cane on precarious terrain. The patient should expect continued use of the AFO. The knee can be extended in a brace by locking it in hyperextension and by increasing the lordotic curve. Patients who discard the brace may fall, fracturing the patella, femur, and/or shoulder. Some patients are uncomfortable with the cosmetic appearance of the thigh. To enhance body image, recommend use of an orthosis or cosmesis fabricated of Pelite to simulate contours of the sound extremity and allow for wearing of contemporary fashions. The orthosis can be suspended by an ACE wrap or held in place with pantyhose.

For patients with soft tissue sarcoma of the medial thigh, an adductor muscle group excision is required. This procedure usually is followed by radiation and chemotherapy. Following this procedure, prolonged drainage through suction catheters is a frequent complication. The patient may require bedrest for long periods of time, sometimes longer than 2 weeks with the obvious resultant sequelae. The lymph nodes are not removed as in a groin dissection, but the medial thigh contains major lymphatic channels that are sacrificed with the specimen. Initially, keep all motion of the extremity to a minimum. Perform loose elastic wrapping to help protect the incision. Isometric contractions of the quadriceps seem to increase drainage when performed as part of an exercise program following surgery to this area and should not be recommended.

Weight bearing, when allowed, is tolerated, but a cane may be necessary for balance. Custom measured elastic stockings or commercial support stockings should be applied for all upright activities. Complaints of motor dysfunction are rare, but edema and pain are common. Educate patients about the importance of leg elevation and avoidance of prolonged sitting. Review techniques of basic skin care, including caution when shaving the legs.

When a tumor is removed from the posterior thigh, tight wound closure may compromise skin in the area of the popliteal fossa. If adjunct radiation and chemotherapy are required, the incision may open and remain a problem for the first year following treatments, despite active participation in physical therapy (PT). PT usually is interrupted and resumed sporadically as complete wound healing progresses. The patient remains in bed, frequently in a knee immobilizer, until drainage subsides. Teach the patient quadriceps isometrics and ankle ROM exercises. When the incision appears to be healing well, start ROM of the hip and the knee. Initiation of knee flexion may be difficult, but this motion can be accomplished in the side lying position. Patients have few physical complaints except for stiffness following prolonged sitting and unsteadiness when running.

Chronic problems that may occur long after medical treatments have been completed are knee flexion and ankle plantar flexion contractures. Institute programs of whirlpool treatments and debridement for slow-healing wounds, serial casting for contractures, and review of stretching exercise techniques. A woman should be discouraged from wearing shoes with excessively high heels. Lateral thigh excisions frequently leave the individual with significant cosmetic and physical deficit, although not so limiting as to prevent normal work or social activities.

Bony tumors involving the proximal tibia or distal femur result in limb-preserving procedures with use of the kinematic rotating hinged knee joint or distal femoral replacement. The incision is long and lateral to the patella. Removal of the distal femur or proximal tibia, along with the joint capsule ligaments and muscle, is necessary. The endoprosthesis maintains skeletal continuity and near-normal function of the knee. Lack of knee stability is inherent. Problems associated with the use of this knee joint in growing children are resolved by use of an expanding or telescoping device.

The patient is placed in a bulky dressing and knee immobilizer in the operating room. Because methyl methacrylate is used to hold the endoprosthesis in place, the dressing is only for control of swelling and comfort. PT can be started as early as day 1 for quadriceps sets, especially for patients with only femoral replacement. Patients with proximal tibial prosthesis are restricted only from vigorous quadriceps function and knee flexion to protect the attachment of the patellar tendon. Recommend that these patients start on gentle active flexion and extension strengthening exercises 4 weeks postoperatively. Some patients with a kinematic rotating hinged knee joint may be placed on continuous passive motion (CPM) machines immediately; however, this alternative has not been found to be more beneficial than an active program.

Potential exists for many complications, such as wound infections, edema, and temporary peroneal nerve palsies from overstretching during surgery. Full active ROM is expected, as well as full weight bearing. The rehabilitation process begins with quadriceps isometrics and progresses until the patient has the ability to ambulate with use of a cane.

Leg muscles are compartmentalized, but not so definitively as muscles in the thigh. Anterior, posterior, and small lateral compartments exist; the interosseus membrane between the tibia and fibula separates the anterior and posterior regions. The anterior compartment actually is more anterolateral and contains muscles that act as dorsiflexors of the foot. If surgical excision is necessary, place the patient in a posterior leg/ankle splint early following surgery to prevent heel cord contracture and overstretching the incision. Place the splint over the surgical dressing for additional protection to the wound and to keep in place while the patient is in bed. Request that the patient use the splint for extended periods even after discharge. For ambulation the patient can use a metal double-upright AFO with dorsiflexion assist or a solid plastic AFO if sensation is intact and there is minimal edema. Use of knee-high support stockings and low-heeled shoes or high-quartered sneakers also is recommended.

Educate the patient about proper foot care. The peroneal nerve and the peroneus longus muscle that evert the foot frequently are sacrificed, either partially or completely. The PT intervention usually consists of heel cord stretching and maintenance of ROM with fitting of a custom-made ankle stabilizer, air splint, or metallic AFO.

The gastrocnemius muscle spans 2 joints and joins with the soleus to form the Achilles tendon, which inserts on the calcaneus. These muscles flex the knee or plantar flex the ankle. The gastroc-soleus complex comprises the posterior compartment of the leg. If radiation is necessary following surgery in the posterior compartment, the knee joint should be spared. Radiation fields generally are directed laterally to spare the skin behind the area. Skin is at high risk of breakdown during treatment.

Secure the lower extremity in a long-leg immobilizer with a posterior splint on the foot. Apply the immobilizer in the operating room to facilitate ease in transferring the patient without disturbing the wound or suction catheters. After 2 weeks, the physical therapist may remove the splints and start gentle ROM to the knee. Partial weight bearing also can be initiated with the knee splint in place. The contralateral shoe may have to be raised temporarily to allow for clearance during swing-through phase of gait.

The complex array of requisite steps for occurrence of this pathologic process suggests multiple potential points of therapeutic antimetastatic intervention. The importance of the appropriate soil also is evident increasingly. Certain cancers have long been known to have a predilection for particular organ distributions of metastasis, such as the tendency to metastasize to bone mentioned above for breast and prostatic cancers. Indeed, different tumors tend to metastasize to specific bony sites, and metastases to distal long bones or small bones of the extremities are extremely rare with most carcinomas. When such acral metastases occur, they typically are associated with lung carcinomas, suggesting some specificity in the metastatic process. Traditional explanations attribute this to anatomic factors (eg, vascular and lymphatic distribution), but recent evidence indicates that local tissue factors may be a strong determinant.

Imaging techniques presently available for detection and monitoring of skeletal metastases include conventional radiography, scintigraphy, CT scan, and MRI. Radiographic skeletal survey now is largely obsolete as a screening method for metastases in patients with malignant disease. Bone scintigraphy is the method of choice in most cases, with the exception of patients with multiple myeloma, in whom bone scintigraphy often produces false-negative results. Conventional radiographs demonstrate a high degree of accuracy in differentiating metastatic bone lesions from primary bone tumors.

The most frequently affected parts of the skeleton are the vertebral column, hip, femur, and humerus. Patterns of bone destruction are recognized and have been described as geographic, moth-eaten, and permeative. Geographic destruction consists of large, well-defined lytic areas greater than 1 cm in diameter with a distinct sclerotic rim. Moth-eaten destruction contains smaller (2-5 mm) lytic areas with ill-defined margins. Permeative lesions associated with bone destruction are characterized by multiple, small (1 mm) areas, principally in cortical bone. Geographic destruction is associated with slow growing tumors, moth-eaten with moderately aggressive lesions, and permeative with highly aggressive tumors. Apart from these lytic lesions, osteoblastic and mixed-type metastases should be recognized radiographically.

The osteoblastic component is not neoplastic, but it should be interpreted as a reaction of normal bone to metastatic cancer. Primary tumors of the prostate and GI tract may account for a blastic response. Lytic lesions frequently are observed in metastases of kidneys, melanoma, and breast and lung tumors. Mixed-type lesions are found in metastases secondary to primary tumors of the breast, GI tract, and reproductive system. Although patients with blastic lesions may have serious problems with bone pain, they tend not to have fractures because of the sclerotic nature of the reactive bone within and around metastatic lesions. The 2 following theories attempt to account for the mechanism of progression of lytic lesions in bone:

1. Direct osteolysis of bone by tumor cells

2. Stimulation of host osteoclastic bone resorption by tumor cells

Recent evidence suggests that both mechanisms may be at work.

Bone scintigraphy is an excellent method for early detection of skeletal metastases, especially in cases where bone lesions remain radiologically occult. Today Tc 99m-labeled polyphosphonates are preferred in bone scans. Bone scans can detect metastatic lesions 2-18 months earlier than conventional radiographs. Multiple myeloma, leukemia, and lymphoma present the most difficulty for the clinician in arriving at diagnosis. Complicating factors in interpretation of bone scans are trauma, infection, and miscellaneous factors, such as preexistent disease (eg, osteoporosis, rheumatoid arthritis). Finding of a lesion in scintigraphy suggests the need for additional evaluation (eg, CT scan, biopsy). Density discrimination in the CT scan is superior to that in conventional radiography. Soft tissue changes are demonstrated well, presenting clinical implications in defining extent and operability of tumors. CT scan also may be helpful in determination of appropriate fields in radiation therapy of metastatic lesions.

In the past, angiography was performed to assess both the vascularity and soft tissue extension of tumors. Enhanced CT scan largely has replaced angiography in this respect; however, angiography and embolization still are important in preoperative assessment and treatment of vascular tumors.

MRI provides soft tissue contrast superior to that of CT scan. Images can be obtained in the axial, coronal, and sagittal planes, permitting clear demonstration of the extent of the lesion, especially the extent of bone marrow involvement. Evidence suggests that MRI is sensitive enough to detect the extent of disease more rapidly than isotope studies can. Remember, however, that MRI study results have to be correlated with other studies.

Clinical history includes a thorough review of symptoms and profound examination of the patient. Bone metastasis often is associated with pain. Discomfort often is worst at night. In extensive bone disease, multiple and migratory areas of pain are recorded. Diagnostic pitfalls include pain in metastatic disease of the spinal column, which is treated as lumbar disc disease, and discomfort around the knee from metastasis in the region of the hip.

Important laboratory studies include analyses of blood, enzymes, proteins, and minerals. Perform serum protein immune electrophoresis routinely to exclude multiple myeloma. Hypercalcemia may be found in patients with bony metastases. Other markers are serum alkaline and acid phosphatase, which is elevated in patients who exhibit large lytic lesions and serum prostate-specific acid phosphatase associated with cancer of the prostate. Carcinoembryonic antigen (CEA) is another indicative test, especially in GI tumors.

Preoperative staging studies also include conventional radiographs, bone scintigraphy, CT scan, and MRI. Consider biopsy to confirm metastatic disease in patients with a known primary tumor, to evaluate a lesion shown on conventional radiographs or on bone scintigraphy, and to obtain tissue for special studies. Prior to performance of biopsy, the surgeon must become aware of the clinical, immunologic, and hematologic condition of the patient. Patients in this group are highly susceptible to infection and hemorrhage. Perform biopsy with great care. Have adequate blood replacement available, for instance, in metastases of carcinoma of the kidney.

Needle aspiration and cytologic evaluation may confirm diagnosis of cancer. In skeletal lesions, however, biopsy is preferred. Fluoroscopic guidance often is useful; a radiograph should be made to document that the correct area has been sampled. Make bacteriologic cultures and frozen sections to rule out infection and to evaluate reliability of the sample. If the pathologist requires special techniques, take appropriate measures. In cases of excessive bleeding, pack the lesion with Gel-foam or polymethyl methacrylate (PMMA). The site of biopsy always should be in line with the definitive incision.

Major progress has been made in surgical management of metastatic skeletal disease over the past 20 years. Many techniques have been developed to treat bone defects. The surgical procedures carried out most frequently in tertiary care hospitals include the following:

• Tumor curettage and cemented osteosynthesis - Intracapsular resection of tumor combined with internal fixation (eg, bone plates, screws, intramedullary rods) and PMMA

• Tumor curettage and spinal instrumentation - Intracapsular resection of tumor followed by interposition of PMMA or some kind of biomaterial (eg, hydroxylapatite), bone grafting, and spinal anterior and/or posterior stabilization

• Tumor resection and joint replacement - Used most frequently in the hip region with PMMA for fixation

• Segmental resection and reconstruction marginal or intracapsular resection of tumor together with a large segment of bone followed by implantation of a custom-made or modular mega endoprosthesis (with PMMA used for fixation purposes) - More common than in the past, especially when reconstruction with ordinary endoprosthesis is impossible because of absence of functional bone remnants

• Cryosurgery - Used to obtain better margins without resection of great amounts of bone; also useful in treatment of tumor hemorrhage

• Amputation - Seldom required; not in agreement with goals of palliative treatment, which should be used in these patients

In addition to this synopsis of different types of treatments available for patients with skeletal defects due to metastases, a focus on several points of interest can be worthwhile.

Bone grafting may be ineffective in promoting bony union unless more than 9 months have elapsed since completion of local radiotherapy, as this is when osteoblastic and chondroblastic properties have been regained. Methyl methacrylate has been shown to provide better fixation, allow earlier ambulation, and not interfere with radiation therapy. The acrylic cement's resistance to compression loads, combined with torque and shear strength of the metallic device, promotes secure fixation.

The object of radiation therapy is to destroy malignant cells in the affected area, facilitate union of fracture, and prevent local recurrence at least for a limited period of time. Radiation therapy produces tumor necrosis and a softening of bone that can increase fracture risk, especially in the first 6-8 weeks; bone does not regain full strength until 6 months after completion of radiation therapy. Over time, stages include degeneration and necrosis of cancer cells, replacement by proliferative fibrous tissue, and aggregation of collagen fiber, which becomes calcified and mineralized, forming bone trabeculae and osteoblastic rimming with woven bone structure maturing into lamellar bone. Recalcification becomes evident within 3-4 months, and normal bone structure may be present 6 months after therapy. Many physicians restrict weight bearing during and up to several months after radiation therapy, but practice varies widely among clinicians.

Primary goals of treatment are relief of pain, restoration of function, and facilitation of nursing care. Remove as much tumor and destroyed bone as possible to eliminate the necessity for a second procedure. Guidelines for treatment of these lesions include risk of failure of fractures to unite, shortened life expectancy of patients, and weakened bone in the vicinity of the tumor. Further criteria for treatment are inadequate reaction of lesion to adjunct therapy, lytic lesion more than 2.5 cm in diameter, and destruction of the cortex exceeding 50% of the circumference.

In case of lesions of the lower extremity permitting partial weight bearing, the rehabilitation physician should be aware of the condition of patients' upper extremities since lesions in these areas may preclude use of walking aids. Nutritional condition of the patient should be optimal. Perioperative antibiotics are obligatory. For vascular lesions, preoperative embolization is advisable. Generally, all patients receive chemotherapy and/or radiotherapy preoperatively and postoperatively to diminish risk of soft tissue seeding and local recurrence. In major bone defects, especially in the hip region, marginal resection always is performed. Choose the plane of resection 3 cm distally or proximally (knee region) of the radiographically recorded boundaries of the tumor. Follow resection by implantation of a mega endoprosthesis with the aid of cement. With this technique, risk for further bone destruction in the area of bone prosthesis interface can be reduced to a large extent.

In the diaphyseal area of long bones, cemented osteosynthesis with an intramedullary (IM) rod and PMMA is preferred to internal fixation with a plate, screws, and PMMA. In the metaphyseal area cemented osteosynthesis after intracapsular resection often is indicated. Provide for extensive vacuum drainage.

Postoperatively, the patient is prescribed bedrest for 24-48 hours. After this period, a rehabilitation program may be initiated. Once drains have been removed, the patient can move from bed to a chair. The patient may commence static resistance exercises and basic ADL training and transfer training, as well as wheelchair mobility training. The patient then can progress to pregait and, subsequently, gait training activities. The aim of this program is to help the patient walk independently with crutches or a walker within 2 weeks postoperatively. Encourage the patient to become independent in transfers and basic ADL with adaptive equipment. Resume radiotherapy and chemotherapy 3 weeks postoperatively. Radiotherapy is an effective method of treatment for cancer and skeletal metastasis.

The primary aim of radiotherapy is relief of pain, restoration of function, and arrest of tumor growth. In patients who exhibit multiple lesions, use radiotherapy for the most symptomatic areas. A total of 30 Gy in 10 fractions, 3 Gy daily, for palliative purposes is recommended. Sometimes a single 8-Gy treatment is given to patients with very short life expectancy. Employ effective chemotherapy and/or hormonal therapy if available. As most patients with bone metastases suffer from breast cancer, recommend empirical treatment with tamoxifen or combination chemotherapy.

Additional treatment with bishosphonates has been proven to prevent a number of events (eg, fractures, need for additional radiotherapy) and may induce sclerosis of lytic bone lesions. Bisphosphonates are effective in treatment of hypercalcemia and can inhibit osteoclast activity by mechanisms that still are unknown. Inhibition of bone mineralization may be a problem in the long run, but not in patients with cancer. Most bisphosphonates are resorbed poorly and should be given on an empty stomach to prevent binding to calcium salts in food. Advise patient to take ample water to prevent local ulceration.

Monitor bone lesions not amenable to surgery and treat with great care. For lesions in the region of the spinal column, prescription of a brace often is justified. Because of tumor progression and effects of radiotherapy, the vertebral body may collapse, and a brace generally prevents excessive axial deviation. Orthosis should immobilize one level above and one below the region of the vertebra with the symptomatic lytic lesion or pathological fracture. Bracing is inadequate to prevent spinal cord compression. Bracing is an excellent ancillary means of metastatic spinal pain management. 

In other cases, such as diaphyseal lesions of the upper extremity, a brace may reduce risk or symptoms of pathologic fracture. A brace may facilitate use of the upper extremity for functional activities that do not involve weight bearing. In lesions of the lower extremity, orthoses may help control pain-related symptoms, but their ability to afford much stability for pathological fractures is limited. If the upper extremities are devoid of significant lytic lesions, achieve restricted weight bearing with an assistive device for ambulation.

Radiotherapy and chemotherapy affect not only the tumor; they also may have adverse effects on adjacent normal bone, causing reduction in the healing potency of bone. In some cases of metastatic disease of the skeleton, physicians use radioactive isotopes to palliate pain.

Prognosis for the patient suffering from skeletal metastasis plays a major role in the therapy concept. In case of short life expectancy, avoid major surgery. Factors contributing to unfavorable prognosis include the following:

• Aggressive primary tumor

• Short recurrence-free interval after primary treatment

• Radiographic absence of bone sclerosis in metastases initially and after systemic therapy

• Multiple bone lesions

• Involvement of more than one organ by metastases (eg, especially the liver)

• High overall tumor burden

• Poor general condition

Survival rate after pathological fracture varies with type of primary tumor. Patients with carcinoma of the lung rarely survive longer than 1 year and often do not survive 6 months, whereas patients with carcinoma of the thyroid commonly live 5 years or longer. In general, approximately 50% of patients sustaining pathologic fracture survive longer than 6 months and approximately 30% survive 1 year. Ability to manage the primary tumor improves through use of chemotherapy, radiation therapy, and surgery. Corresponding increase in postfracture survival time necessitates improved surgical methods and development of implants to provide better management for these patients.

Factors contributing to a more favorable prognosis include the following:

• Moderately progressive primary tumor (prostate cancer)

• Long recurrence-free interval after primary treatment

• Radiographic presence of sclerosis in bone metastases initially and after systemic treatment

• Solitary bone lesion, preferably of a geographic type

• Low overall tumor burden (preferably bone only)

• Good general condition of the patient

Few QOL studies exist for patients with metastatic skeletal disease. Clohisy et al found the SF-36 to have questionable value in identifying patient characteristics that yielded higher QOL scores. Heterogeneity of the patient population and floor effects limited utility of the SF-36 in his cohort of patients. The Functional Living Index - Cancer (FLIC) was somewhat more helpful. Scores of the subscale for physical well-being at 6 weeks correlated with increased length of survival.

Goals of rehabilitation include relief of pain and improved ambulation and function. A paucity of literature exists on effectiveness of traditional inpatient rehabilitation measures for patients with malignancy involving bone. These individuals often sustain significant loss of mobility and have much to gain empirically from treatment. Bunting et al studied 58 patients with 62 pathologic fractures at various bony sites. Average length of rehabilitation stay, 37 days, was only slightly higher than that for general postfracture patients.

Functional results were mixed, with 26 patients achieving independent transfers, 23 independent ambulation, and 27 improved scores for ADL. A total of 34 patients were discharged to home and 7 to other facilities. Mortality was high; 17 patients died. Hypercalcemia and the need for parenteral narcotics were risk factors for death or poor result from rehabilitation. In a separate study, Bunting et al found that risk of fracture during PT among patients on an oncology unit was low, only 1 patient of 54; however, 12 patients did sustain fractures during hospitalization (circumstances were not described).

In these patients, imaging studies show cortical atrophy and hyperdense white matter changes. Although pathogenesis of these alterations is unknown, high-dose/large-fractionation schedules may be a factor. Therefore, in patients with anticipated long survivals, a longer course of radiotherapy with smaller doses per fraction probably is indicated. A reasonable schedule for patients with good prognosis would be a total dose of 4500 to 5000 cGy given in daily fractions of no more than 200 cGy.

Although advantages to surgery can be cited for selected patients, WBRT alone remains the treatment of choice for most patients with brain metastases. Approximately 33% of patients, and, unfortunately, nearly 50% of patients in this group are not surgical candidates because of inaccessibility of the tumor, extensive systemic disease, and other factors. Therefore, only about 15-20% of all patients with brain metastases benefit from surgical resection; treat the rest with radiotherapy.

Stereotactic radiosurgery, a method of delivering intense focal irradiation using a linear accelerator (eg, LINAC) or multiple cobalt-60 sources (eg, gamma knife), also has been used to treat single and multiple brain metastases. Radiosurgery delivers a highly focused single dose of radiation to a circumscribed area of the brain. The technique causes tissue destruction in the area targeted; therefore, radiosurgery does not replace whole-brain radiotherapy, but it may offer a substitute for surgical therapy.

Radiosurgery is limited to lesions smaller than 3 cm in diameter. Many uncontrolled studies of highly preselected patients have been published. Combined results of several reports suggest that radiosurgery prevents (or controls) local recurrence of 80-90% of treated metastases with about 5-10% risk of radiation necrosis or new neurologic deficits. Prospective clinical trials currently underway are expected to help determine the role of radiosurgery, both in primary treatment of patients with single metastases and in management of recurrent brain metastases.

Use of interstitial brachytherapy, a technique involving placement of radioactive implants within the area of the tumor, has been advocated in selected patients. Implants allow delivery of high-dose focal radiation to the tumor, while minimizing risk of significant radiation exposure of surrounding normal brain tissue because of rapid fall off in radiation intensity at margins of the precalculated target area. The procedure is limited to relatively small metastases located in surgically accessible regions of the brain.

Preliminary studies suggest that brachytherapy may be effective in selected patients with brain metastases. The major complication of brachytherapy is radiation necrosis, which may present as an expanding mass appearing months after treatment. Biopsy often is required to differentiate tumor necrosis from recurrence; steroids and, occasionally, surgical resection help to reverse neurologic symptoms secondary to radiation necrosis. Frequency of this complication varies with the amount of radiation administered.

Along with radiosurgery, brachytherapy may be an additional treatment option for patients with unresectable metastases or prior maximal doses of WBRT; however, the role of brachytherapy in management of brain metastases has yet to be determined.

Chemotherapy has been used in treatment of brain metastases from a variety of primary tumors; however, results generally have been unimpressive. Although some small uncontrolled studies of patients with certain highly chemosensitive tumors (eg, breast, small cell lung cancer, germ cell tumors) have been published, chemotherapy is not usually the primary therapy for most patients, and it is seldom the only therapy. Based on presently available data, reasonable use for chemotherapy for brain metastases is in patients with small, asymptomatic tumors known to be chemosensitive. If progression occurs with administration of chemotherapy alone, more definitive treatment with surgery or radiation may be indicated.

Accumulating evidence suggests that chemotherapy may have a role in treatment of carefully selected patients with brain metastases; however, efficacy of chemotherapy in management of brain metastases has not been demonstrated conclusively. Unfortunately, most systemically administered chemotherapeutic agents proven effective against systemic cancer have been ineffective against cerebral metastases from the same cell population.

All patients need careful follow-up care, no matter what type of initial treatment they receive for brain metastases. Unfortunately, no standard has been set for frequency of follow-up neuroimaging studies after treatment. Clearly, MRI or CT scans are indicated any time after therapy if patients develop new neurologic symptoms.

For patients treated with surgery, contrast MRI scans should be performed within 5 days of surgery to detect residual disease. These tests are important, especially if consideration is being given to forego postoperative radiation therapy. Obviously, if residual disease is present, patients should be given WBRT. For all patients treated with WBRT, follow-up scans should be obtained at regular intervals after treatment. In general, it takes about 6 weeks after WBRT for definite change to take place in the scan, so patients usually do not need scans immediately following completion of radiotherapy. A reasonable schedule of follow-up scanning would be to obtain a scan 2 months after completion of the last therapy (either WBRT or surgery) and then about every 3-4 months for the first year following treatment. The length of time between scans then can be extended gradually so that asymptomatic patients undergo scanning procedures only once per year.

Rehabilitation issues

The Meyers and Boake survey of 30 caregivers of patients with brain tumors provides a clear pattern of concerns that appears to differ from concerns of other medically ill populations. The most salient problems facing patients with brain tumors were lack of energy; inability to perform usual activities around the home (ie, paying bills, making repairs); social isolation; lack of sexual activity; general slowing of behavior; and problems with reasoning, memory, and concentration. In contrast to other medically ill populations, caregivers of patients did not endorse certain problems with brain tumors as being worthy of concern. These nonproblems included depression, ability to perform basic ADL (eg, dressing, eating), ambulation, and ability to speak and be understood. Thus, it appears that neurobehavioral problems have the largest impact on QOL of patients with tumors and their families.

Metastatic disease of the spinal column

Most patients with systemic cancer develop skeletal metastases, and the spine is involved most commonly. Spinal metastases are present in 40% of patients who die of cancer. Autopsy studies have shown that distribution of spinal metastases parallels the bulk of the vertebrae; thus, the lumbar spine most often is affected, followed by the thoracic and cervical segments. Clinically, however, symptomatic spinal metastases most often involve the thoracic spine (in 70% of cases), followed by the lumbar (20%) and cervical segments (10%).

Secondary spinal tumors most often originate from primary tumors of the breast, lungs, and prostate, reflecting both prevalence of these cancers and their propensity to metastasize to bone. Ten percent of patients with symptomatic spinal metastases present with no known primary lesion.

Symptomatic spinal metastases produce a characteristic clinical syndrome beginning with pain, followed by weakness, sensory loss, and sphincter dysfunction. Local back or neck pain is the earliest and most prominent feature in 90% of patients. Palpation or percussion over the posterior spine at the affected level usually elicits local tenderness. Associated radicular pain distribution indicates irritation of segmental root(s). When movement aggravates local back or neck pain, which then is relieved by immobility, suspect spinal instability. If pain has a severe, burning, or dysesthetic quality, then intradural extramedullary metastases are the likely cause. Pain caused by spinal metastases may be present for up to 1 year and often is attributed initially to arthritis, back strain, or slipped disc. An acute onset of back or neck pain in a cancer patient means spinal metastasis until proven otherwise.

Pain may be present for up to 1 year (average of 4 months) before more blatant manifestations of spinal cord compromise manifest. Rates at which spinal cord compression develops vary; however, once established, weakness, sensory loss, and sphincter dysfunction progress to complete and irreversible paraplegia unless timely treatment is undertaken.

MRI is the imaging modality of choice for spinal tumors, including spinal metastases. The spine may be evaluated in various planes, and the entire spinal column can be visualized in sagittal cross-sections. Patterns of extradural metastases can be identified, including an isolated level of focal disease, multiple levels of contiguous involvement, or multiple levels of disparate tumor foci. MRI with gadolinium enhancement permits identification of typical intradural extramedullary "drop" metastases typically found along the cauda equina nerve roots and also reveals any intramedullary metastases. Coronal, sagittal, and transverse reconstructions from MRI provide important information concerning location and geometry of secondary spinal tumors and demonstrate integrity of adjacent vertebral bony elements, all of which are essential for planning optimal treatment.

Treatment

Corticosteroids alleviate pain acutely and improve neurologic function; administer promptly to patients with clinical manifestations of metastatic epidural compression confirmed by diagnostic imaging or when strongly suspected on clinical grounds, pending confirmation by diagnostic imaging. Dosage of dexamethasone, the most commonly reported corticosteroid used, remains controversial. While some authors recommend 4 mg qid, laboratory studies have shown a dose-related benefit with dexamethasone leading to clinical use of loading dose of 100 mg followed by 24 mg qid. A common approach uses 10-100 mg dexamethasone IV stat, followed by 4-24 mg qid. The larger doses are reserved for patients with profound or rapidly progressive neurologic injury and lower doses for patients with mild or equivocal signs.

Steroid administration usually is continued throughout radiation therapy at a tapering dose. One useful tapering schedule is approximately one third reduction in dose every 3-4 days (eg, from 16 mg to 12 mg to 8 mg). A trial of escalated dose followed by taper may be attempted if tapering is not tolerated and neurologic deterioration occurs. Consider the toxicities of high-dose corticosteroids.

Decisions regarding the level of therapeutic intervention involve factors relative to prognosis, as well as the extent of disease in the spine. Radiation therapy is the treatment of choice for most cases of spinal cord compression because no overall difference in neurologic outcome has been observed when patients are treated either by radiation therapy or surgery plus radiation therapy. Response to radiation therapy alone is reported to be 80%. This rate of response with radiation therapy alone represents improvement of motor dysfunction in 49% and stabilization of clinical status in an additional 31% of cases.

Extent of epidural mass, like paravertebral involvement, influences response to radiation because of the size of the tumor and presenting neurologic injury. Patients presenting with complete spinal cord block generally have greater residual neurologic impairment after radiation therapy than those with partial block because of irreversible spinal cord injury. Neurosurgical intervention is indicated to establish a diagnosis; remove a tumor that is compressing the spinal cord, resulting in neurologic symptoms and/or intractable pain; or treat disease that persists or recurs in a previously irradiated area. Surgical intervention often is used in radioresistant tumors like melanoma and for stabilization of the spine.

Parameters of response involve pain and functional status. These parameters may influence and/or predict survival. Aranzio et al found that pretreatment functional status is maintained in more than 90% of surviving patients for average follow-up period of 3 years after radiation therapy. More than 40% of patients who could ambulate before and after radiation therapy for spinal cord compression survived for 1 year, and 20% of this group survived for 3 years after treatment. In contrast, only 30% of patients who were nonambulatory at presentation and 7% of nonambulatory patients were alive at 1 year after treatment.

Techniques used to treat spinal cord compression with radiation account for factors of radiation dose and treatment volume. Although a variety of radiation treatment schedules is used, the most common is 3000 cGy administered in 10 treatments (300 cGy per treatment) to the area of the spinal cord compression. Radiobiologically, this regimen is approximately equivalent to administering 3600 cGy using 200 cGy per treatment in a conventional radiation schedule. Using a more abbreviated course of radiation is considered advantageous in patients who have severe pain because they often have other intervening medical problems. If long segments of the spine are involved by tumor or if radiation can exit through viscera like the stomach, 3500 cGy is administered in 14 treatments to improve patient tolerance; this radiation schedule delivers a dose to the spinal cord that is radiobiologically equivalent to 3850 cGy if administered at 200 cGy per treatment.

The radiation portal typically is 8 cm wide, centered at the midline of the spine. Generally, the radiation portal includes the area of spinal cord compression, plus a margin of 2 vertebral levels above and below the region involved by metastatic disease.

Hyperfractionation is a radiation treatment schedule that exploits the radiobiologic principle of repair of normal tissues between radiation fractions. Two small radiation doses are given on each treatment day; typically a minimum interval of 6 hours separates each dose of 100 to 120 cGy per fraction. During the hour interval between radiation doses, normal tissues undergo repair of the radiation effects. This process of repair of normal tissues allows safe administration of higher total doses of radiation to normal tissues such as the mucosae and skin; however, repair of damage to the spinal cord is lower, and experiments have shown that it takes more than 8 hours for the spinal cord to complete repair of radiation injury. Radiation tolerance of the spinal cord is reduced by 0-15% when the interval between radiation actions is decreased from 24 hours to 6-8 hours. Unlike with the skin and mucosae, hyperfractionation does not spare the spinal cord from radiation injury.

Radiation-induced myelopathy results from dosage to white matter and vasculopathies, which represent 2 separate mechanisms of radiation injury. Damage to white matter is associated with diffuse demyelination and swollen axons that can be focally necrotic and have associated glial reaction. Vascular damage has been shown experimentally to be age dependent and can cause hemorrhage, telangiectasia, and vascular necrosis.

Six major types of injury have been shown experimentally to result from radiation to the spinal column. Five of these occur in the spinal cord and one in the dorsal root ganglia. The most severe spinal lesions, all of which are from vascular damage and result in neurologic dysfunction, include white matter necrosis, hemorrhage, and segmental parenchymal atrophy. Clinically, these types of lesions occur 12-50 months after x-ray therapy (XRT). They can manifest as Brown-Séquard syndrome or as transverse myelitis.

The extent of neurologic loss runs the gamut from incomplete to complete. The 2 less severe spinal lesions include focal fiber loss and scattered white matter vacuolation resulting from damage to glial cells, axons, and/or the vasculature. These less severe sequelae are seen with lower total doses of radiation and are less likely to result in neurologic dysfunction. In dorsal root ganglia, radiation damage includes intracytoplasmic vacuoles and loss of neurons and satellite cells, which can affect sensory function. These findings are distinct from demyelination of the posterior columns associated with the self-limiting Lhermitte syndrome. This pattern of spinal cord injury occurs early on during the first 12-20 weeks after XRT and usually resolves within 1 year.

Meningeal thickening and fibrosis also can be observed after irradiation, but the clinical significance is unknown. Ependymal and nerve root damage from radiation is rare. Recent refinement in surgical strategies, including elaboration of the posterolateral approach and anterior avenues for spinal decompression, together with evolution of spinal stabilization procedures, have improved outcome for patients undergoing surgery for spinal metastases. This, in turn, has lent support to the concept of de novo surgery for secondary spinal tumors. Furthermore, operating on tumors minimizes risk of wound complications primarily, which can be as high as 30% with surgery through an irradiated tissue bed.

Indications for surgery in patients with symptomatic spinal metastases include the following:

• Failure of radiation therapy

• Uncertain diagnosis

• Pathologic fracture dislocation

• Rapidly progressing or far advanced myelopathic symptoms and signs

Prompt initiation of radiation therapy is imperative to maintain neurologic integrity. At present, pretreatment deficits resulting from spinal cord compression are the most important predictors for posttreatment function. Overall, fewer than 50% of patients regain lost functional capacity. More than 95% of patients with lung cancer and spinal cord compression retain ability to ambulate, but only 19% regain ability to ambulate after radiation therapy for epidural disease. Normal bladder function is maintained in 30% of patients, but approximately 50% of patients require catheterization both before and after radiation therapy. Recovery of neurologic function also depends on the rate of progression of symptoms, radiosensitivity of the tumor, and extent of epidural mass. Response is more likely in paraparetic patients with radiosensitive tumors, such as multiple myeloma, and germ cell and lymphoproliferate tumors.