38 - Management of Perioperative Cardiac Events
Editors: Shields, Thomas W.; LoCicero, Joseph; Ponn, Ronald B.; Rusch, Valerie W.
Title: General Thoracic Surgery, 6th Edition
Copyright 2005 Lippincott Williams & Wilkins
> Table of Contents > Volume I - The Lung, Pleura, Diaphragm, and Chest Wall > Section IX - The Chest Wall > Chapter 46 - Chest Wall Tumors
Chapter 46
Chest Wall Tumors
Bernard J. Park
Valerie W. Rusch
Chest wall tumors arise from a wide variety of benign and malignant etiologies and provide the clinician with a diagnostic and therapeutic challenge. More than half are malignant, and most of these are the result of metastases or direct invasion from adjacent tumors of the thorax, mediastinum, or soft tissue. Primary chest wall tumors are rare; of these, soft tissue tumors account for roughly 50%. Wide surgical resection is the most effective treatment for the vast majority of chest wall tumors. Improvements in reconstructive techniques and care of the perioperative patient have led to low morbidity and mortality rates for chest wall resection. Keys to successful management include accurate diagnosis, wide surgical resection, and appropriate reconstruction of large chest wall defects. The optimal treatment of locally recurrent breast cancer and metastatic lesions to the chest wall is controversial.
CLASSIFICATION AND INCIDENCE
Chest wall tumors fall into one of four categories: primary tumors; adjacent tumors with local invasion; metastatic lesions; and nonneoplastic disease (Table 46-1). The relative incidence of each of the categories varies depending on the series examined, but the majority of chest wall lesions are the result of metastasis or invasion from adjacent malignancies. Pairolero and Arnold (1985) reported the Mayo Clinic experience with 100 consecutive patients who underwent chest wall resection, and demonstrated that metastatic lesions (32%) and adjacent tumors (24%) together accounted for 56% of all chest wall lesions. Similarly, Martini and coauthors (1987) reported a 20-year experience of 317 consecutive patients undergoing chest wall resection for malignant tumors at Memorial Sloan-Kettering Cancer Center. Primary lung and breast cancer made up 163 cases (51%), while 71 patients (22%) had metastatic lesions. In both series, sarcomas were the most frequent metastatic tumors.
Primary chest wall tumors, by comparison, are relatively uncommon, representing roughly 5% of all thoracic neoplasms and 1% to 2% of all primary tumors, as reviewed by Incarbone and Pastorino (2001). In the Mayo series, 44% of all chest wall tumors were primary neoplasms, whereas only 83 (26%) of the 317 chest wall lesions in the Memorial experience were primary tumors.
Benign tumors account for approximately half of all primary chest wall neoplasms, with rates ranging from 21% to 67% in reports by Groff and Adkins (1967), Teitelbaum (1972), and Stelzer and Gay (1980), as well as by Sabaratnam (1985), Graeber (1982), and King (1986) and their associates. The most frequently encountered benign lesions are osteochondroma, chondroma, and fibrous dysplasia.
Like benign neoplasms, primary malignant tumors arise from the soft tissue or the bony and cartilaginous elements of the chest wall. As demonstrated by Burt (1994), soft tissue sarcomas make up 45% of all primary malignant lesions. Desmoid tumor, liposarcoma, and rhabdomyosarcoma are the most frequent histologies of sarcoma encountered. Chondrosarcoma, Ewing's sarcoma, and osteosarcoma are the most common malignant bone lesions and are the most common primary malignant chest wall tumors overall in our experience.
Chest wall neoplasms, primary and metastatic, can arise from or involve any portion of the thoracic skeleton, including the ribs, sternum, scapulae, and clavicles. The most common site of involvement for all chest wall lesions is the rib cage. Primary lesions of the sternum, scapulae, and clavicles are uncommon, but nearly all are malignant, as reported by Dahlin and Unni (1986). Malignant primary chest wall neoplasms can also originate in a previously irradiated field, although this is a relatively uncommon event. Schwarz and Burt (1996) showed that 6% of patients (21 of 351) with primary chest wall malignancies had received prior irradiation. There is no predominant histologic cell type, with the usual spectrum of lesions occurring.
Table 46-1. Classification of Chest Wall Tumors | |
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DIAGNOSIS AND EVALUATION
Signs and Symptoms
Soft tissue chest wall tumors are generally asymptomatic initially, presenting as slowly enlarging masses. As Gordon and colleagues (1991) reported, 73% of patients with primary soft tissue sarcomas of the chest wall presented with a nonpainful mass. With continued growth and tumor involvement of surrounding tissue, pain invariably occurs. The pain is frequently generalized, and the patient commonly is treated for musculoskeletal or neuropathic etiologies. Almost all malignant lesions are likely to become painful, whereas only two-thirds of benign tumors produce pain. Cartilaginous and bony tumors more frequently have pain as one of the first symptoms of disease. Burt (1994) showed that pain with or without a mass was present in 49% of patients with chondrosarcoma, in 95% with Ewing's sarcoma, and in 78% with osteosarcoma. In some cases with rib lesions, a radiographic abnormality can be discovered with no mass palpable on physical examination. Occasionally, there may be systemic manifestations, such as fever, leukocytosis, and eosinophilia.
Diagnosis
The initial evaluation of any patient suspecting of having a chest wall neoplasm begins with a complete history and physical examination. The clinician should pay particular attention to any history of prior malignancy, exposure to ionizing radiation, or presence of familial conditions, such as Gardner's syndrome or von Recklinghausen's disease. Standard conventional plain chest radiographs should be obtained and compared with any previous studies, if available. Computed tomography (CT) of the chest is the single best radiographic modality to localize and characterize most chest wall tumors in order to generate a differential diagnosis and treatment strategy. In some instances, magnetic resonance (MR) imaging of the chest can add useful information. As reported by Wyttenbach (1998) and Fortier (1994) and their co-workers, compared with CT, MR imaging can better delineate the extent of muscular invasion, relationship to nearby blood vessels, and nerve or spinal involvement by chest wall lesions. CT, however, is superior in the assessment of calcifications and presence of pulmonary pathology. Although specific tumors may have features better elucidated by one or the other study, it is clear that these imaging modalities are complementary. Moreover, as Schaefer and Burton (1989) emphasized, while judicious use of imaging and careful analysis of films can often lead to a correct diagnosis, radiographs tend to be most useful in the planning of tissue biopsy or surgical resection.
Following the history, physical examination, and imaging, if there is doubt whether a chest wall neoplasm is benign or malignant or if the lesion is suspected of being a primary tumor, tissue diagnosis by histologic examination is required. Biopsy should be well planned, minimally traumatic to the primary tumor and surrounding tissue, and diagnostic. There are three options: needle biopsy, excisional biopsy, and incisional biopsy.
Needle Biopsy
The benefits of needle biopsy include minimal soft tissue injury, ease of performance, and low complication rate in an
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outpatient setting. Fine-needle aspiration (FNA) alone is best employed in patients who are suspected of having a metastasis from a prior documented malignancy. It is not as good for situations where a larger amount of tissue is required for pathologic diagnosis, such as a primary bony or cartilaginous chest wall tumor. Percutaneous core needle biopsy is a viable option for the diagnosis of primary chest wall tumors and is a well-accepted technique for biopsy of primary bone tumors. Welker and coauthors (2000) demonstrated a diagnostic rate of 92.6% in 161 musculoskeletal tissue masses suspected of being sarcoma.
Excisional Biopsy
Excisional biopsy with minimal margins (1 cm) should be employed for small (less than 5 cm) primary neoplasms. Closure of the chest wall usually can be performed primarily without reconstruction. If the lesion is benign or is best treated with nonsurgical therapy (chemotherapy, radiation therapy, or both), then no further operation is required. If the lesion is a primary malignant neoplasm, the patient should undergo reoperation with radical excision with wide margins and appropriate reconstruction.
Incisional Biopsy
Incisional biopsy should be performed when a needle biopsy is nondiagnostic or for primary lesions larger than 5 cm. It is important to orient the skin incision to facilitate its excision at the time of definitive resection and to promote optimal wound healing, avoiding ulceration and infection that might delay therapy. Skin flaps should not be raised, and the deep plane of the tumor, in particular the pleural space, should be left intact to prevent dissemination of tumor cells.
Evaluation
Although advances in surgical technique, anesthesia, and perioperative management have reduced morbidity and mortality, extensive chest wall resection and reconstruction remains a major surgical procedure. As with other thoracic operations, careful preoperative assessment is critical to minimize the risks to the individual patient. In addition to a detailed history and physical examination, this evaluation should include a thorough investigation of cardiopulmonary comorbidities, smoking history, performance status, and psychosocial support. The presence of cardiovascular disease, such as coronary artery or valvular disease, has important implications for intraoperative and postoperative hemodynamic monitoring and fluid management. The authors are in the practice of routinely obtaining cardiac stress testing in patients over the age of 70, in particular if they have a long history of smoking, hypertension, diabetes mellitus, or other manifestations of atherosclerotic disease (peripheral vascular disease, carotid stenosis, or aneurysm). If the patient's pulmonary reserve is of concern, it should be assessed with pulmonary function tests that include spirometry, diffusion capacity, and arterial blood gas analysis. Patients with chronic obstructive pulmonary disease (COPD) or other active pulmonary disease should have optimal medical treatment prior to surgery.
Preoperative consultation with a variety of subspecialists is often necessary. For instance, in the case of malignancies such as Ewing's sarcoma and plasmacytoma, evaluation by medical and radiation oncologists prior to operation is an integral part of the patient's multimodality treatment. Depending on the size of the lesion, chest wall reconstruction may warrant the use of myocutaneous flaps, a technique that is best performed in conjunction with plastic and reconstructive surgeons. Similarly, as reported by McCormack and associates (1981), neurosurgical consultation is often required for tumors encroaching on the spine. When complete resection is limited by proximity of the lesion to vital structures, external beam radiation therapy (either preoperatively or postoperatively) or intraoperative brachytherapy is often employed at our institution, as described by Wallner and colleagues (1991).
SURGICAL MANAGEMENT
In suitable patients, surgical resection for cure is appropriate for primary chest wall neoplasms. In select cases of metastatic lesions and locally recurrent breast cancer, surgical extirpation of an isolated chest wall lesion can result in long-term survival benefit. Even in instances where the likelihood of cure is low, chest wall resection may be indicated to palliate lesions unresponsive to nonsurgical therapies (chemotherapy, radiation therapy) and lesions that are causing local complications, such as wound ulceration, infection, or intractable pain.
The margin of excision during definitive resection for chest wall lesions varies somewhat depending on the type of neoplasm. Moreover, what constitutes an adequate margin of excision for a primary malignant chest wall tumor to minimize the incidence of recurrence and maximize chances for long-term survival is a matter of some debate. What is clear is that the extent of resection should not be limited to the size of the resulting defect. With a wide variety of techniques available, bony and soft tissue deficits of most any size can be reconstructed with excellent results.
If the overlying skin is involved, Chapelier and co-workers (1994) recommend excision of at least 0.75 cm of normal skin. Previous scars from incisional biopsies and irradiated skin, if present, should be included in the resection. If the tumor does not involve the skin or subcutaneous tissues, the incision can be placed directly over the mass, and flaps may be utilized for primary closure. However, when there is surrounding soft tissue involvement, a full-thickness resection must be performed. As previously described
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by El-Tamer and coauthors (1989), we advocate inclusion of one normal musculofascial plane between the skin and lesion, sparing uninvolved muscles that can be used for reconstruction.
The pleural cavity is entered one intercostal space below or above the involved rib, and the extent of the tumor in the chest is evaluated. Any attached structures, such as lung, thymus, or pericardium, should be resected en bloc. The recommended cephalad and caudad margins of resection are one uninvolved rib each. The optimal lateral margins are somewhat controversial. If the lesion is a benign, metastatic, or low-grade malignant primary neoplasm (e.g., chondrosarcoma), most surgeons would agree that a resection margin of 2 to 4 cm around the macroscopic tumor, as described by McCormack and associates (1989), is acceptable. For the majority of primary malignant lesions, the possibility of tumor spread within the marrow or along tissue planes, such as the periosteum or parietal pleura, mandates wider resection. The authors remove a minimum of 4 to 5 cm of grossly normal tissue, with microscopic confirmation of negative margins by frozen section (Fig. 46-1). Pairolero and Arnold (1985) advise excision of at least 4 cm of normal tissue on all sides of all primary malignant lesions previously diagnosed by excisional biopsy. In the Mayo Clinic experience, as analyzed by King and colleagues (1986), the 5-year recurrence-free rate was 56% for patients resected with a 4-cm margin compared with 29% for those resected with a 2-cm margin. Moreover, for high-grade lesions involving the lateral chest wall, such as malignant fibrous histiocytoma, Pairolero (2000) advocates removal of the entire ribs involved, including the corresponding anterior costal arches if the tumor is anteriorly located, and several partial ribs above and below the tumor.
For tumors of the sternum, the bony resection is started over the costal margins after the extent of invasion of mediastinal structures is determined. The affected portion of the sternum should be removed with a 2- to 3-cm rim of surrounding costochondral cartilage and rib. Again, if involved, segments of parietal pleura and pericardium should be included in the resection. Whenever possible, it is preferable to keep a portion of the sternum intact for stability and reconstruction.
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Fig. 46-1. Diagrammatic representation of the preferred extent of resection for chest wall tumors. The resection margin should include one normal rib cephalad and caudad to the tumor and 4 to 5 cm of grossly normal tissue laterally. From El-Tamer M, Chaglassian T, Martini N: Resection and d bridement of chest-wall tumors and general aspects of reconstruction. Surg Clin North Am 69:947, 1989. With permission. |
SPECIFIC TUMORS
Primary Bone Tumors
Primary bone neoplasms of the chest wall are uncommon. Dahlin and Unni (1986) summarized a series of 6,034 bone tumors, of which 355 (5.9%) occurred in either the ribs (85%) or the sternum (15%). Nearly all were malignant (89%), with sternal tumors slightly more likely to be malignant than rib tumors (96% versus 88%). The most common benign bone lesions are osteochondromas, chondromas, and fibrous dysplasia. Martini and co-workers (1987) reported that fibrous dysplasia accounts for 30% of benign tumors of the chest wall. The most common primary malignant bone tumors seen at Memorial Sloan-Kettering Cancer Center, as described by Burt (1994), were chondrosarcoma, Ewing's sarcoma, and osteosarcoma.
Osteochondroma
Although osteochondroma is overall an uncommon tumor, it is the most common benign bone neoplasm, constituting nearly 50% of all benign rib tumors. It arises from the bony cortex in the metaphyseal region of a rib and develops as a stalked mass with a cartilaginous cap. Microscopically, the amount of bony proliferation and thickness of the cartilaginous cap varies. Stippled calcification is often present within the tumor, and a rim of calcification can be present at the periphery (Fig. 46-2). The tumor begins in childhood and continues to grow until skeletal maturity is reached. The lesion may grow inward, remaining asymptomatic, or grow outward, producing a palpable mass.
Osteochondromas detected in children after puberty or in adults should be resected. Before puberty, resection is indicated for the onset of pain or increase in size, especially because malignant degeneration has been reported. Men are affected three times more frequently than women. Complete surgical resection is the treatment of choice. Recurrence has never been reported.
Chondroma
Chondromas represent 15% of all benign rib neoplasms, and most commonly occur in the second or third decade of life, affecting both sexes equally. They present as slowly enlarging, asymptomatic masses originating anteriorly at the costochondral junction. The radiographic appearance is that of an expansile, medullary mass that causes thinning of
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the cortex. Clinically and radiographically, it is not possible to differentiate chondroma from chondrosarcoma. Histologically, the tumor consists of lobules of hyaline cartilage. However, the microscopic differentiation between a chondroma and a low-grade chondrosarcoma can be difficult. Therefore, all chondromas must be managed as malignant lesions, with wide excision to prevent local recurrence.
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Fig. 46-2. A 52-year-old man with osteochondroma arising in the anterior right ninth rib. Note the intact cortex and stippled calcification within the tumor. |
Fibrous Dysplasia
Fibrous dysplasia is a benign, cystic lesion characterized by fibrous replacement of the medullary cavity of the rib. It accounts for up to 30% of all benign chest wall tumors and most commonly presents as a solitary mass in the lateral or posterior rib cage. Both sexes are equally affected, and multiple lesions can occur in Albright's syndrome (multiple bone cysts, skin pigmentation, and precocious sexual maturity in girls). These lesions are slow growing and remain asymptomatic unless they become large enough to cause symptoms through local compression or pathologic fracture. The radiographic findings are characteristic: a trabeculated, expansile lesion with a ground-glass center and thinning of the cortex. Microscopic examination reveals bony trabeculation, fibrous tissue, and, occasionally, calcification. Malignant degeneration is unusual, and treatment should be conservative. Resection is curative and is indicated to rule out malignancy and for painful, enlarging lesions.
Eosinophilic Granuloma
Eosinophilic granuloma is not a neoplasm, but one subset of a spectrum of diseases of the reticuloendothelial system that fall under the moniker of histiocytosis X. This also includes Letterer-Siwe disease (infants) and Hans-Sch ller-Christian disease (children), both of which are characterized by systemic signs and symptoms in addition to bony lesions. These include fever, malaise, weight loss, lymphadenopathy, and splenomegaly associated with leukocytosis, eosinophilia, and anemia. Eosinophilic granuloma is limited to bone involvement and typically occurs in young to middle-aged adults. The radiographic appearance of the bone abnormality is similar for all three: an expansile lesion with periosteal new bone formation and uneven destruction of the cortex that produces endosteal scalloping. The skull is most commonly involved, but 10% to 20% of patients have rib lesions. Excisional biopsy is required for diagnosis, and is curative in patients with solitary eosinophilic granuloma. Patients with multiple lesions should undergo low-dose radiation therapy. In the systemic forms of the disease, the course tends to be protracted and requires treatment with chemotherapy and corticosteroids.
Malignant Rib Lesions
Chondrosarcoma
Chondrosarcoma is the most common primary chest wall bone neoplasm and accounts for one-third of all primary malignant bone lesions. In over 60% of all cases, chondrosarcoma of the chest wall typically arises in the costochondral arches of rib or sternum. Occurring more frequently in men, it is rare before the age of 20 years and usually occurs in the third or fourth decade of life. The exact cause is unknown, and most chondrosarcomas arise de novo, although malignant degeneration of benign cartilaginous tumors has been reported. Lichtenstein (1977) and McAfee and coauthors (1985) have described an association between trauma and chondrosarcoma.
Patients develop a slowly enlarging mass that eventually becomes painful. Radiographically, the tumor appears as a lobulated mass arising in the medullary portion of the bone, with destruction of the cortex and mineralization of the tumor matrix that produces a mottled type of calcification (Fig. 46-3). Microscopically, findings can range from normal cartilage to obvious malignant changes with plump, atypical, and multiple nuclei that may be more apparent in the periphery of the tumor (Fig. 46-4). Definitive diagnosis can only be made pathologically and often requires abundant tissue in order to distinguish a well-differentiated chondrosarcoma from a chondroma.
The treatment of chondrosarcoma at any site is complete resection. Analyzing our results at Memorial Sloan-Kettering Cancer Center, Burt and associates (1992) reported an
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overall 5-year survival of 64%, with a median survival of 148 months. In a univariate analysis, the factors associated with prolonged survival were lack of metastases, complete resection, lack of recurrence, and age less than 50 years (Table 46-2). Gender, site of primary tumor, size, and grade were not prognostic. McAfee and colleagues (1985) found an overall 10-year survival rate of 53.4% in the Mayo experience, demonstrating that the extent of resection, tumor size, and tumor grade each had a significant effect on survival. By multivariate analysis, extent of resection and tumor grade remained the most important prognostic factors. The 10-year survival rate for patients with chondrosarcoma treated with wide resection (28) was 96.4%, versus 65.4% (p < 0.048) for those treated with local excision (25). Patients with grade I lesions had a 70.8% 10-year survival, compared with 39.2% for patients with grade II and 0% for patients with grade III tumors (p < 0.0001).
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Fig. 46-3. CT scan of a 34-year-old woman with a large chondrosarcoma arising from the anterolateral left fifth rib. Note the mottled calcification within the mass. |
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Fig. 46-4. Photomicrograph demonstrating the typical microscopic features of chondrosarcoma. |
Table 46-2. Prognostic Factors in Primary Chest Wall Chondrosarcoma | ||||||||||||||||||||||||||||||||||||||||||||||||
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Ewing's Sarcoma
Ewing's sarcoma is a small round-cell sarcoma that occurs primarily in flat bones and the midshaft of long bones. Primary Ewing's sarcoma of the chest wall represented 15% of all Ewing's sarcomas and 17% of all primary malignant chest wall tumors treated at our institution over a 40-year period. Two-thirds of these cases occurred in patients younger than 20 years, and boys and men were affected twice as often as girls and women. Almost all patients presented with a painful, enlarging mass associated with fever, malaise, leukocytosis, anemia, and an increased erythrocyte sedimentation rate (ESR). The radiographic features can mimic osteogenic sarcoma, osteomyelitis, or other bone tumors. Mottled destruction containing lytic and blastic areas appears, and elevation of the periosteum and multiple layers of subperiosteal new bone formation can cause an onion-skin appearance of the bony surface. Pathologic fractures are uncommon. Histologically, the tumor is cellular and must be distinguished from other small round-cell tumors such as lymphoma, embryonal rhabdomyosarcoma, and metastatic neuroblastoma, among others.
In the Memorial series reported by Burt and co-workers (1993), 23% of patients had synchronous metastases at presentation, and 71% of patients who presented with only local disease developed distant metastases. For this reason, Ewing's sarcoma should be considered a medical tumor, with the surgeon's initial role being to establish a diagnosis
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either by incisional or core needle biopsy. Following systemic chemotherapy, the primary site should either be irradiated or resected. Using a combined-modality treatment, the overall 5- and 10-year survival rate at our institution was 48%, with a median survival of 57 months. More recent multicenter trials of induction chemotherapy followed by resection, radiation therapy, or both, published by Shamberger (2000) and Sirvent (2002) and their coauthors, demonstrated 5-year event-free survival rates of 62% to 68%.
Osteosarcoma
Osteosarcoma of the bony thorax is less common than chondrosarcoma, constituting 10% of all primary malignant chest wall tumors in our 40-year experience and 3% of all osteosarcomas. It is, however, a more virulent neoplasm, with a poor prognosis. It generally occurs in teenagers and young adults, with a slight predilection for boys and young men. Most patients present with a rapidly enlarging, painful mass and elevated serum alkaline phosphatase levels. Bone destruction with indistinct borders that merge into adjacent normal bone appears on radiographs (Fig. 46-5). Calcification occurs at right angles to the bony cortex, producing a sunburst appearance. Pathologic fractures are rare. The tumor grossly is large, lobulated, and extends through the cortical bone into the adjacent soft tissue. Microscopically, the predominant component may be bony, cartilaginous, or fibrous.
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Fig. 46-5. A. Chest radiograph of a 49-year-old woman with an osteosarcoma arising from the lateral right tenth rib. B. CT scan of the same patient. |
In our series of patients, 34% had synchronous metastases, and resection was the primary therapy in 82%. Adjuvant chemotherapy was used in 48% of resected patients. Over two-thirds of the patients (68%) developed distant metastases at some point in their course. The overall 5-year survival was 15%, with a median survival of 12 months. No patient who developed distant metastasis survived 5 years. Approximately half of the resected patients received adjuvant chemotherapy, yet they enjoyed no improvement in survival. Our current approach is to give patients induction chemotherapy followed by wide resection.
Solitary Plasmacytoma
Solitary plasmacytoma to a rib is uncommon, representing only 6% of all primary chest wall malignancies and 3% of all plasmacytomas seen in 40 years at Memorial Sloan-Kettering Cancer Center. Although solitary plasmacytomas may present in the absence of systemic disease, 75% ultimately progress to multiple myeloma. Myeloma is most common in the fifth through seventh decades of life, and two-thirds of those afflicted are men. Pain is the most common symptom, often without an associated mass. Most patients are anemic and have an elevated ESR. Abnormal protein electrophoresis is present in 85%, and up to 50% have urinary Bence Jones protein and hypercalcemia. Radiographically, myeloma appears as an osteolytic lesion with cortical thinning. Pathologic fracture is common. Histologically,
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sheets of closely packed, hyperchromatic, multinuclear cells with abundant cytoplasm and rare mitoses are seen.
The majority of our patients (67%), as reported by Burt (1994), received primary chemotherapy followed by either resection (one third) or radiation therapy (two thirds). The overall 5- and 10-year survival rates were 38% and 21%, respectively, with a median survival of 56 months. Because most patients presenting with a solitary plasmacytoma of the chest wall subsequently develop multiple myeloma, the primary role of the surgeon is to establish a diagnosis either by core needle, incisional, or excisional biopsy. As described in most modern series reported by Chak (1987), Dimopoulos (1992), Frassica (1989), and Holland (1992) and their associates, the standard primary treatment modality for solitary plasmacytoma is radiation therapy. The major prognostic factor for survival is the development of multiple myeloma.
Primary Soft Tissue Tumors
Benign Tumors
Primary soft tissue tumors may arise from any cell type of the thorax and may be benign or malignant. The predominant benign tumors involving the chest wall include fibromas, lipomas, giant cell tumors, neurogenic tumors, vascular tumors (hemangiomas), and connective tissue tumors. Neurogenic tumors involving the bony thorax include neurilemomas and neurofibromas. As reported by Martini and colleagues (1987), neurofibromas can occur as isolated lesions or in association with von Recklinghausen's disease (neurofibromatosis). Neurilemomas usually occur as solitary tumors that resemble neurofibromas but are not associated with neurofibromatosis. Although sarcomatous degeneration may occur in neurofibromas and, to a lesser extent, in neurilemomas, malignant degeneration of benign lesions overall is uncommon, and all can be treated by local excision.
Malignant Soft Tissue Tumors
Malignant soft tissue lesions and, in particular, soft tissue sarcoma account for approximately 50% of all primary malignant chest wall tumors. Preoperative differentiation between the various neoplasms, however, can be difficult. When in doubt, as with bony lesions, wide resection of tumor with surrounding structures is the preferred treatment.
Desmoid
Desmoid tumors are locally invasive tumors with a propensity to recur following resection. As described by Posner and co-workers (1989), they arise most commonly in extremity locations (42% to 51%). The chest wall has been reported as a site of origin in 10% to 28% of patients. Gordon and associates (1991) showed that desmoids represented the most common primary chest wall sarcoma, making up 21% of the histologic subtypes treated at Memorial Sloan-Kettering Cancer Center. Desmoid tumors affect males and females equally, most frequently between adolescence and 40 years of age, and can be associated with Gardner's syndrome. The tumor originates in muscle and fascia, extending along tissue planes and displacing and often encasing surrounding structures, including vessels (Fig. 46-6). Initially asymptomatic, desmoids involving the thoracic inlet can cause paresthesias, hyperesthesia, and motor weakness with progressive neural encasement. Histologically, there is a spreading pattern of uniform, well-differentiated fibroblasts and fibrocytes with an abundant intercellular matrix without mitoses or necrosis. Frequently, there are fingerlike projections of tumor infiltrating the surrounding tissue well beyond the gross extent.
Based on these microscopic findings, and because desmoids do not tend to metastasize, some, such as Goellner and Soule (1980) and Hayry and coauthors (1982), consider them to be benign fibromatoses. Others, however, consider desmoid tumors to be malignant, low-grade fibrosarcomas because of their aggressive local invasion, propensity to recur, and consequent morbidity. Desmoid tumors should be treated with wide surgical resection. Death from disease following resection is rare, but local recurrence is common. Brodsky and associates (1992) reviewed our institution's experience, reporting an overall 5-year survival rate of 93% and a 5-year local recurrence rate of 29% following resection. On univariate analysis, the only factor associated with a higher risk of recurrence was age greater than 30 years at the time of diagnosis. We have employed intraoperative brachytherapy when wide resection is limited by tumor proximity to vital structures. Recurrence should be treated, when feasible, with repeat resection. Patients with multiply recurrent tumor should be considered for adjuvant radiation therapy. Options for treatment of patients in whom surgery is not feasible include hormonal therapy, nonsteroidal antiinflammatory medication, and chemotherapy.
Soft Tissue Sarcoma
Although soft tissue sarcomas of the chest wall account for one half of primary malignant chest wall lesions, and the majority of metastatic lesions as well, they are still relatively uncommon. Burt (1994) reported that primary chest wall sarcomas represented only 6% of all soft tissue sarcomas seen over a 40-year period at Memorial Sloan-Kettering Cancer Center. Gordon and colleagues (1991) showed that men are affected twice as often as women. The majority of primary chest wall sarcomas occur in adult life, with the exception of rhabdomyosarcoma, which is seen most frequently in children and young adults before the age of 45 years. Most patients present with a painless mass, and a wide range of histologic subtypes is seen (Table 46-3).
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Fig. 46-6. A. CT scan of a 21-year-old woman with a locally recurrent, extensive desmoid tumor of the chest wall. B. MR image of the same patient. |
Table 46-3. Primary Soft Tissue Sarcoma of Chest Wall: Histologic Subtypes in 149 Patients | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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As with soft tissue sarcomas in other sites, primary chest wall sarcomas are generally treated primarily with wide surgical resection. In our series of 149 patients reported by Gordon and co-workers (1991), 140 patients were treated primarily with resection, with 70 undergoing resection at our institution. Local recurrence developed in 59% overall and in 27% of those who had their resection at Memorial Sloan-Kettering Cancer Center. Neither margin status nor grade of tumor was associated with a higher incidence of local recurrence. Fifty-one percent of patients with high-grade sarcoma developed metastases, compared with only 10% of patients with low-grade histology. Overall 10-year survival was 56%, with a significant difference in survival between high-grade disease (39%) and low-grade disease (82%), between patients who developed metastases (19%) and those who never developed metastases (75%), and between those with pain at presentation (37%) and those with no pain (63%) in high-grade sarcomas.
Because of higher rates of recurrence, metastases, and poorer overall survival in high-grade chest wall sarcoma, most cases are approached with multimodality therapy. Perry (1990) and Walsh (2001) and their coauthors are among several authors who have published series in which these lesions were treated with a variety of combined modalities that included induction therapy, surgical resection, and adjuvant therapy. Induction and adjuvant therapies included chemotherapy alone, radiation therapy alone, or chemoradiation therapy. The overall 5-year survival rates approximate 60%. One form of soft tissue sarcoma that is sensitive to chemotherapy is rhabdomyosarcoma. This fact, coupled with an otherwise dismal survival with resection alone (21% 10-year survival), has resulted in current treatment regimens that include induction chemotherapy, complete surgical excision, and adjuvant therapy with radiation therapy, chemotherapy, or both, as pointed out by Ryan and associates (1989).
Radiation-Associated Malignant Tumors
In the last 25 years there have been four series describing postirradiation chest wall sarcomas. The two largest, by Souba and colleagues (1986) and Schwarz and Burt (1996), reported rates of 4.8% and 6% for all primary chest wall sarcomas, respectively, reflecting the uncommon nature of this problem. The most common indications for radiation therapy were for breast carcinoma or lymphoma. In the study by Schwarz and Burt (1996), the median latency period between irradiation and development of a primary chest wall sarcoma was 7 years (range 2 to 19), and although a wide range of histologies was observed, osteosarcoma made up over one-half of the 21 lesions. The median dose of radiation therapy was 4,140 cGy (range 1,250 to 9,500), with most patients receiving external beam radiation. The precise mechanism of carcinogenesis for postirradiation sarcomas is not known. The results of treatment of lesions arising in a previously irradiated field were comparable to their de novo counterparts, with similar survival rates following wide surgical resection. Thus, these patients should be treated in a manner commensurate with that typically employed for the de novo pathologic tumor type.
Tumors of the Sternum, Clavicle, and Scapula
Primary chest wall tumors (benign and malignant) and metastatic lesions can frequently involve the manubrium
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and sternum. Benign lesions are typically chondromas, bone cysts, or hemangiomas. Metastatic tumors usually originate from carcinomas of the breast, thyroid, or kidney. In the Memorial Sloan-Kettering Cancer Center series summarized by Burt and co-workers (1992), the sternum was the site of origin in 14% of bony and cartilaginous tumors overall. Chondrosarcoma is the most common histologic type, followed closely by osteosarcoma, plasmacytoma, and lymphoma. As Pairolero and Arnold (1985), as well as Martini (1996) and Incarbone (1997) and their associates, showed in the largest published series, complete surgical resection via partial (<50%), subtotal, or total sternectomy with repair of defects employing rigid prostheses is a safe and effective treatment. Overall survival following complete surgical resection is related to the tumor histologic type and grade.
The scapula is a common site for primary bone tumors, but is an infrequent site for metastatic lesions. As Burt and colleagues (1992) noted, the scapula was the site of origin for malignant bony and cartilaginous lesions in 31% of cases overall. The most common histologies include chondrosarcoma, Ewing's sarcoma, and osteosarcoma, all of which occur with equal frequency. Wide surgical resection is the treatment of choice.
In contrast, with the exception of plasmacytoma, the clavicle is an uncommon site for primary malignant bony tumors, accounting for the site of origin in only 8% of cases. In fact, the clavicle is more likely to be a site of metastatic disease than of primary tumor. Most clavicular lesions are malignant and should be treated with primary radiation therapy or complete surgical resection. Reconstruction following resection is generally unnecessary.
Metastatic Lesions and Recurrent Breast Carcinoma
The role of surgical resection for metastatic disease to the chest wall or locally recurrent breast carcinoma is controversial because the former reflects disseminated disease and the latter portends it. As previously mentioned, metastatic disease accounts for 20% to 30% of all chest wall neoplasms and can occur within the bony thorax or the soft tissues surrounding the ribs, sternum, scapula, or clavicle. The majority cannot be cured by surgical extirpation, but resection can be considered in certain cases for cure and in others to palliate pain or ulceration and infection of the overlying skin. Criteria for curative resection, as proposed by Anderson and Burt (1994), include the following:
the chest wall is the only site of disease
locoregional disease is controlled
complete resection with negative margins is possible
Martini and co-workers (1987) reported a 20% 5-year survival for chest wall metastasectomy, and 41% of patients were alive at a median follow-up of 31.5 months in the series by Pairolero and Arnold (1985).
Up to 10% to 12% of patients with stage II breast carcinoma experience a local recurrence after mastectomy, sometimes with chest wall involvement. As demonstrated by Valagussa and coauthors (1978), the majority recur within the first 3 years, and up to 80% of patients with locoregional recurrence will develop distant disease. Disease isolated to the chest wall and a disease-free interval from mastectomy to recurrence longer than 2 years correlate with survival following surgical resection of local chest wall recurrence. McCormack and associates (1989) reported a 50% 5-year disease-free survival in 35 patients who underwent chest wall resection. As reviewed by Incarbone and Pastorino (2001), a number of small series show 5-year survival rates that range from 35% to 58% following curative resection. Aggressive treatment of local failure, in addition to the potential curative benefit, results in palliation of pain, removes a potentially unsightly wound, and achieves optimal local control of disease.
CONCLUSIONS
Chest wall tumors provide a diagnostic and therapeutic challenge to the thoracic surgeon. Successful management depends on timely diagnosis, careful patient evaluation, and aggressive surgical resection with adequate chest wall reconstruction where appropriate. With modern surgical and reconstructive techniques, this procedure can be performed in a single operation with minimal pulmonary compromise and low operative morbidity and mortality. Moreover, in the majority of primary chest wall tumors, complete and wide surgical resection can lead to potential disease-free and overall survival. In the case of certain histologic subtypes, multimodality therapy combining chemotherapy, surgical resection, and radiation therapy in both induction and adjuvant settings is appropriate. Surgical management of metastatic tumor and recurrent breast carcinoma is often necessary for palliation, and in isolated cases can lead to prolonged survival.
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