CHEST WALL TUMORS
Introduction
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Tumors of the chest wall include a wide variety of bone and soft tissue diseases ( Tables 50-1 and 50-2 ):
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Primary benign and malignant neoplasms of the bony skeleton and soft tissue
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Neoplasms that invade the chest wall from adjacent structures such as the lung, pleura, mediastinum, and breast
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Postradiation-induced chest wall neoplasms, which are frequently necrotic
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Non-neoplastic benign disorders
TABLE 50-1 ▪
CLASSIFICATION OF CHEST WALL TUMORS
Primary chest wall neoplasms
Malignant
Benign
Metastatic chest wall neoplasms
Carcinoma
Sarcoma
Adjacent neoplasms with local chest wall invasion
Breast
Pleura
Lung
Benign non-neoplastic disorders
Cyst
Inflammation
Adapted from Pairolero PC: Tumors of the chest wall. In Niederhuber JE, editor: Current Therapy in Oncology. New York: Mosby, 1993. p. 218–221.
TABLE 50-2 ▪
PRIMARY CHEST WALL TUMORS
Malignant
Bone and Cartilage
Askin’s tumor
Chondrosarcoma
Ewing’s sarcoma
Lymphoma
Myeloma
Osteogenic sarcoma
Soft Tissue
Hemangiosarcoma
Leiomyosarcoma
Liposarcoma
Lymphangiosarcoma
Malignant fibrous histiocytoma
Neurofibrosarcoma
Rhabdomyosarcoma
Benign
Bone and Cartilage
Aneurysmal bone cyst
Chondroma
Chondroblastoma
Eosinophilic granuloma
Fibrous dysplasia
Giant cell tumor
Osteoblastoma
Osteochondroma
Soft Tissue
Desmoid
Fibroma
Hemangioma
Lipoma
Lymphangioma
Neurilemoma
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Thoracic surgeons are frequently asked to evaluate and manage patients with chest wall neoplasms for
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Establishment of diagnosis
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Treatment for cure
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Management of necrotic, foul-smelling ulcerated tumors
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From a practical standpoint, wide surgical resection is the preferred treatment for the majority of chest wall tumors. The following items are important to successful surgical resection:
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Establishment of a correct diagnosis
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Ability to reconstruct large chest wall defects
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Incidence
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Primary chest wall tumors including both bony and soft tissue neoplasms comprise approximately 5% of all thoracic tumors and 1% to 2% of all primary tumors found in the body. The incidence of malignancy in primary chest wall tumors varies ranging from 50% to 80%. The malignancy rate is higher for soft tissue neoplasms compared with bony or cartilaginous tumors.
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In a Mayo Clinic series of 100 consecutive patients having chest wall resection for tumor Pairolero and Arnold found
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Local invasion from adjacent tumors in 24%
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Metastatic lesions in 32%
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Primary chest wall tumors in 44%
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In a 20-year experience involving 317 patients with resected chest wall tumors, Martini and colleagues from Memorial Sloan-Kettering Cancer Center found:
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Primary lung cancer in 127 patients (40%)
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Metastatic carcinoma (breast, kidney, colon, other) or sarcoma in 107 patients (34%)
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Primary chest wall sarcoma in 83 patients (26%)
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The most common primary malignant chest wall tumors the thoracic surgeon is asked to manage are chondrosarcoma, Ewing’s sarcoma, liposarcoma, malignant fibrous histiocytoma, osteosarcoma, and rhabdomyosarcoma.
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The most common benign chest wall tumors the thoracic surgeon is asked to manage are chondroma, desmoids, fibrous dysplasia, and osteochondroma.
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Desmoids are controversial tumors that are considered to be benign by some because of their microscopic features and the fact that they do not distantly metastasize. However, others consider desmoids low-grade fibrosarcomas because of their locoregional extension and propensity for recurrence.
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Any portion of the thoracic skeleton (clavicles, ribs, sternum, and scapula) may be involved with primary or metastatic chest wall tumors.
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The most common site of involvement is the ribs.
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In a review of 11,087 Mayo Clinic cases, Unni and coworkers found primary neoplasms of the sternum, clavicle, and scapula were rare, but when present, the overwhelming majority were malignant.
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Clinical Presentation
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In general, patients with benign chest wall tumors are 14 years younger (average age 26 years) when compared with patients with malignant tumors (average age 40 years).
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The male-to-female ratio for chest wall tumors is approximately 2:1, except for desmoids, which in some series have a 1:2 female-to-male ratio.
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Chest wall tumors commonly present as asymptomatic, slowly enlarging masses.
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Some are incidental findings on chest imaging obtained for unrelated reasons.
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Presenting symptoms are chest wall masses (70%) and pain (25-50%).
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As they grow, nearly all malignant chest wall tumors become painful compared with only two thirds of benign tumors.
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The pain is initially generalized, and many patients have a history of treatment for musculoskeletal complaints or neuritis.
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Fatigue, fever, weight loss, lymphadenopathy, brachial plexus neuropathy, leukocytosis, and eosinophilia may be present.
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Diagnosis
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Successful treatment requires early diagnosis and, in many cases, surgical resection with appropriate reconstruction.
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The diagnostic evaluation should include
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Careful history and physical examination with particular attention to personal histories of malignancy, ionizing radiation, and trauma ( Figs. 50-1A and B )
Figure 50-1
A, A 56-year-old gentleman with a giant chondrosarcoma arising from the right anterior upper costochondral arch. He had a distant history of significant right chest wall trauma. B, Chest x-ray study.
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Laboratory tests include liver function tests and alkaline phosphatase levels.
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Conventional x-ray studies of the involved area may be useful, and if possible, comparison with prior studies may help in assessing the rate of growth ( Figs. 50-2 and 50-3 ).
Figure 50-2
Fifty-three year old gentleman with chondrosarcoma of the right anterior sixth rib. The mass had been present for 18 months without pain.
Reproduced from McAfee MK, Pairolero PC, Bergstralh EJ, Piehler JM, Unni KK, McLeod RA, et al. Chondrosarcoma of the chest wall: factors affecting survival. Ann Thorac Surg 1985;40:535–540, with permission.
Figure 50-3
A 32-year-old woman with chondrosarcoma of the left anterior first rib. The mass had been present with pain for 2 months.
Reproduced from McAfee MK, Pairolero PC, Bergstralh EJ, Piehler JM, Unni KK, McLeod RA, et al. Chondrosarcoma of the chest wall: factors affecting survival. Ann Thorac Surg 1985;40:535-540, with permission.
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Computed tomographic (CT) scans help in the delineation of soft tissue, bony, pleural, mediastinal, and pulmonary involvement. CT is superior to magnetic resonance imaging (MRI) in the assessment of calcification ( Fig. 50-4 ) and possible pulmonary metastases. Although MRI is not routinely obtained, it can be used selectively and is helpful in delineating blood vessels, nerves, and spinal cord involvement.
Figure 50-4
Computerized tomography of a 52-year-old gentleman with a right anterior chest wall chondrosarcoma. Of note is the destruction of the bony cortex and calcific stippling throughout the neoplasm.
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Although CT and MRI are capable aiding in the differentiation of chest wall neoplasms, their sensitivity and specificity are not accurate enough to eliminate the need for tissue diagnosis.
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Whether or not to biopsy a chest wall tumor depends upon the clinical presentation and suspected histopathology.
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Biopsy techniques include
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Fine-needle aspiration (FNA), which often yields a cytology specimen not sufficient for the diagnosis of primary chest wall neoplasms.
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Core needle biopsy, which yields a specimen that maintains the neoplasm’s architecture and in recent years has demonstrated an accuracy of 92% to 96%
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Excisional biopsy, which if performed, should include a minimum 1-cm margin. Excisional biopsy may be appropriate for small 1- to 5-cm neoplasms. The location of the excisional biopsy must not interfere with possible future wide surgical resection. Chest wall closure for these small neoplasms is usually straightforward and does not require skeletal reconstruction. For benign neoplasms, no further treatment is needed. For malignant neoplasms, the treatment may be chemotherapy, radiation therapy, wide surgical resection, or a combination of all three.
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Incisional biopsies should be approached cautiously because of the concern over local tumor spread and the possibility of underdiagnosis. Incisional biopsies may be appropriate when a needle biopsy is nondiagnostic, when metastatic disease from a known distant primary is suspected, or for larger (>5 cm) primary neoplasms. Incisional biopsies must be via small, carefully oriented incisions placed so that definitive wide resection if eventually required is not compromised. Skin flaps should not be raised, the pleura not broached, and the capsule of the mass closed following biopsy.
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If the chest wall mass is suspected of being infectious, then FNA may be helpful in establishing a diagnosis.
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Chest wall tumors suspected of being metastatic from a known primary elsewhere can be accurately diagnosed by FNA, core needle, or incisional or excisional biopsy techniques.
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In our practice, core needle and incisional biopsies are not routinely performed for suspected primary benign or malignant chest wall neoplasms.
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Needle and incisional biopsies may underdiagnose certain low-grade malignancies such as chondrosarcoma, misleading the surgeon not to perform wide resection, which, in turn, compromises the patient’s opportunity for cure.
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The author, Pairolero, and Unni believe that chest wall neoplasms suspected of being metastatic from a known primary elsewhere can be accurately diagnosed with FNA, core needle, or incisional biopsies (Peter C. Pairolero, MD, and K. Krishnan Unni, MD, personal communication, 2008).
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The author and Pairolero believe that either benign or malignant primary neoplasms are preferably diagnosed with an excisional biopsy, which includes a minimum 1 cm margin (Peter C. Pairolero, MD, personal communication, 2008). The location of the excisional biopsy should not interfere with subsequent treatment. Excisional biopsy results in
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Excision of the entire mass, thus providing adequate tissue to firmly establish the tumor’s histopathology
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At Mayo Clinic Rochester, frozen section gives us an immediate, accurate and definitive diagnosis in greater than 90% of bone tumors (K. Krishnan Unni, MD, personal communication, 2008).
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Histopathologically proven benign neoplasms need no further treatment.
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If the neoplasm is best treated by chemotherapy, radiation, or both, then no additional surgical resection is required.
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If frozen section diagnosis cannot be established at the time of excisional biopsy then the chest wound should be closed. If malignancy is eventually diagnosed that is best treated by wide resection, then subsequent wide resection including en bloc resection of the entire biopsy site (skin, subcutaneous tissue, and muscle) is performed (Peter C. Pairolero, MD, personal communication, 2008).
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At the University of Texas M.D. Anderson Cancer Center, core needle biopsies are attempted in all patients, and there have been no incisional biopsies required for the diagnosis of chest wall neoplasms since the late 1980s.
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Pathology
Benign Bone/Cartilage Tumors
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Osteochondroma
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Most common benign bone neoplasm, representing almost 50% of all benign rib tumors
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Begins in childhood and grows until skeletal maturity is reached. Affects men 3 times more frequently than women
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Most patients are asymptomatic; however, pain may indicate malignant degeneration.
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Arises from the bony cortex in the metaphyseal portion of the rib and looks like bony protuberance on a stalk with a cartilaginous rim
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Radiographically stippled calcifications may be seen within the tumor as well as a calcified rim.
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Treatment of choice is resection.
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Complete surgical resection is recommended in children after puberty and in adults. In children before puberty, osteochondromas should be resected for pain or increasing size.
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Chondroma
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Fifteen percent of all benign rib neoplasms
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Can occur at any age, with both sexes equally affected
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Presents as an asymptomatic, occasionally slightly painful anterior costochondral junction mass
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Radiographically seen as an expansile mass with thinning of the cortex
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Grossly it is a lobulated mass, and microscopically lobules of hyaline cartilage are seen.
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Cannot clinically or radiographically differentiate a chondroma from a low-grade chondrosarcoma. Microscopic differentiation may also be very difficult
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All chondromas must be considered malignant and thus managed with wide excision of 2 to 4 cm.
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Fibrous dysplasia
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A cystic, non-neoplastic lesion probably representing a developmental bony abnormality characterized by fibrous replacement of the medullary cavity of the rib.
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Usually presents as a slowly enlarging, solitary, nonpainful mass in the posterolateral rib cage.
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Both sexes are equally affected.
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Radiographically it has a characteristic appearance consisting of an expansile lesion with thinning of the bony cortex and a central ground-glass appearance. Microscopically, bony trabeculations, fibrous tissue, and occasional calcification are seen.
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Although treatment can be conservative, resection is often undertaken for painful, enlarging lesions, and to rule out malignancy. Resection is curative.
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Eosinophilic granuloma
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A disease of the lymphoreticular system and not a true neoplasm
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May be solitary or multifocal and is the unifying feature of the conditions designated as histiocytosis X (eosinophilic granulomas, Letterer-Siwe disease, and Hans-Schüller-Christian disease).
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Occurs in young to middle-aged adults, with peak incidence of 5 to 15 years
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Most commonly found in the skull, but rib involvement is seen in 10% to 20% of afflicted patients.
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Presentation is often pain limited to the involved bone or bones, but patients with Letterer-Siwe and Hans-Schüller-Christian disease may have systemic findings including fever, malaise, weight loss, lymphadenopathy, and splenomegaly, anemia, eosinophilia, and leukocytosis.
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Radiographically an expansile rib lesion is seen with periosteal new bone formation. Uneven destruction of the bony cortex results in endosteal scalloping.
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Microscopically there are eosinophils, giant cells, Langerhans cells, and neutrophils.
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In patients with eosinophilic granuloma alone, excision is diagnostic and curative if the lesion is solitary. For patients with multiple eosinophilic granulomas, the treatment is low-dose radiation therapy (300 to 600 cGY). For Letterer-Siwe and Hans-Schüller-Christian disease, systemic treatments including chemotherapy and corticosteroids are used.
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Benign Soft Tissue Tumors
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A variety of benign neoplasms of the chest wall have been reported. These include fibromas, hemangiomas, lipomas, lymphangiomas, and neurogenic tumors. Malignant degeneration is rare, and all can be treated by local excision.
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Desmoid
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Desmoid tumors are rare soft tissue tumors whose etiology is not completely understood and have a propensity for local recurrence.
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Forty percent occur in the shoulder and chest wall regions.
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Thirty-three percent of patients with Gardner’s syndrome have desmoid tumors, and 2% of patients with desmoids have Gardner’s syndrome.
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Most commonly occur between adolescence and 40 years of age, and are only rarely seen in infants and the elderly.
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Reported to be associated with a history of trauma and estrogen-induced growth.
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Initial presentation is a poorly circumscribed mass with little or no pain. With growth of the tumor, pain may occur. Desmoids located in the shoulder region cause entrapment of the brachial plexus, resulting in paresthesias, hyperesthesia, and motor weakness. This also limits the margin of resection and increases the risk of local recurrence.
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They originate in muscle and fascia extending along tissue planes often with finger-like projections into the surrounding tissues ( Fig. 50-5 ).
Figure 50-5
Computerized tomography of a 19-year-old woman with a right posterolateral lower chest wall desmoid ( arrow ).
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Microscopically, there is a uniform bland spreading pattern of fibroblasts without mitoses or necrosis but infiltration of the surrounding tissues occurs. Some pathologists consider these benign fibromatosis and others low-grade fibrosarcoma.
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Treatment is wide surgical resection.
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We reported on 53 patients who underwent resection of chest wall desmoid tumors. Five-year overall probability of developing a recurrence was 37.5%. Recurrence occurred in eight of nine patients (89%), with positive resection margins and eight of 44 (18%) with negative margins. Factors adversely affecting the rate of postoperative recurrence were the need for reoperation, positive resection margins, proximity to the brachial plexus, and postoperative radiotherapy. Importantly, patients who had postoperative radiotherapy were more likely to have undergone an incomplete resection. None of our patients who had negative surgical margins and subsequently had postoperative radiotherapy had recurrent tumor, which is in contrast to almost 33% of patients who did not have radiotherapy and had recurrence. Importantly, however, whether postoperative radiation therapy is beneficial in all patients who have complete resection of their desmoid cannot be determined from our nonrandomized data. Others have demonstrated radiotherapy to be of benefit in patients with gross residual disease or recurrence.
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Tamoxifen has been reported to decrease both the size and symptoms of desmoid tumors and may be of benefit in patients not capable of having surgical resection.
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Malignant Bone/Cartilage Tumors
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Chondrosarcoma
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Accounts for 30% of all primary chest wall bony neoplasm and occurs most frequently in the anterior chest wall, with 75% arising in the costochondral arches or sternum.
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More common in men
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Most common in middle age
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In the Mayo Clinic series by McAfee and colleagues, the median age was 54 years, and ranged from 17 to 78 years.
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Cause is unknown.
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Malignant degeneration of benign cartilaginous tumors has been reported.
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Association with severe crushing ipsilateral chest wall trauma was found in 12.5% of patients in McAfee’s series.
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Presentation commonly is a slowly enlarging, painful mass.
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Radiographically there is a lobulated mass arising in the medullary portion of the rib or sternum with cortical destruction. A stippled calcification pattern is most common (see Fig. 50-4 ).
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Because the majority of chondrosarcomas are well differentiated, differentiation from chondroma is difficult, and misdiagnosis of chondroma is common. A definitive diagnosis can only be made pathologically, and it is for this reason that excisional biopsy is recommended.
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Pairolero states that from a practical standpoint, all tumors arising within costal cartilage should be considered malignant and, thus, treated by wide resection.
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Natural history is one of slow growth, local recurrence, and if untreated, late metastases.
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Treatment is complete wide resection because these tumors are not sensitive to chemotherapy or radiation.
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Surgical resection margins should be 4 cm in all directions
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Wide resection results in cure in nearly all patients. The 10-year survival rate for patients with chondrosarcoma treated by wide resection was 96%, local excision 65%, and palliative excision 14% ( P < 0.0001) ( Fig. 50-6 ).
Figure 50-6
Survival of 72 patients with chondrosarcoma classified by the extent of resection. Zero time on the abscissa represents the day of resection. The numbers in parentheses are the number of patients alive at 5, 10, and 15 years.
Reproduced from McAfee MK, Pairolero PC, Bergstralh EJ, Piehler JM, Unni KK, McLeod RA, et al: Chondrosarcoma of the chest wall: factors affecting survival. Ann Thorac Surg 1985;40:535-540, with permission.
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Although in McAfee’s series tumor diameter, grade, location, and date of surgical resection all effected survival, a more recent review from our institution showed that the only prognostic factor was the surgical margin.
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Osteosarcoma
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Osteosarcoma is the most frequent bone tumor; however, chest wall osteosarcomas are less common than chondrosarcomas, constituting 6% of all primary malignant chest wall bone neoplasms.
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They are a more malignant tumor with a worse prognosis than chondrosarcoma.
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Occur most commonly between ages of 10 and 25 years and after age 40.
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There is an association between osteosarcomas and prior radiation therapy, Paget’s disease, and chemotherapy. Souba and colleagues found that the mean latency period from radiation therapy was 13 years and the prognosis for radiation-induced sarcomas of the chest wall is very poor.
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Frequently presents as a rapidly enlarging painful mass.
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Serum alkaline phosphatase levels and lactate dehydrogenase levels may be elevated and correlate with decreased survival.
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Radiographically osteosarcomas can be large, lobulated, and extend through cortical bone into adjacent soft tissues ( Fig. 50-7 ). Calcifications may occur producing a sunburst appearance.
Figure 50-7
Computerized tomography of an osteosarcoma of the right third rib with bone destruction.
Reproduced from Graeber GM, Jones DR, Pairolero PC: Chest wall and sternum: primary neoplasms. In Pearson FG, Cooper JD, Deslauriers J, Ginsberg RJ, Hiebert CA, Patterson GA, Urschel HC Jr, editors. Thoracic Surgery. 2nd ed. Philadelphia: Churchill Livingstone; 2002. p. 1417–1430, with permission.
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Treatment is neoadjuvant chemotherapy in order to shrink the tumor before resection.
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The combination chemotherapy of cisplatin, doxorubicin, and high-dose methotrexate is most common.
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Surgical resection consists of wide resection of the tumor, including the entire involved bone (rib or sternum) and adjacent soft tissues.
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Radiation therapy has not been of benefit.
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With the addition of multidrug neoadjuvant chemotherapy, 5-year disease-free survival rates have improved from 20% with surgery alone to greater than 50%.
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Ewing’s sarcoma
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Account for 12% of all primary chest wall bony neoplasms.
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Sixty-six percent of all Ewing’s sarcomas occur in persons younger than 20 years of age; however, it is rare in infants.
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Presentation commonly is a progressively painful enlarging mass. Fever, malaise, anemia, leukocytosis, and an elevated erythrocyte sedimentation rate may be present.
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Radiographically, an onion-skin appearance of the bone caused by elevation of the periosteum and multiple layers of new subperiosteal bone formation may be seen, but this is not pathognomonic. These features can be seen in osteosarcoma, osteomyelitis, and other bone tumors.
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Usually, Ewing’s sarcoma is confined to one rib; however, involvement of multiple ribs does occur ( Fig. 50-8 ).
Figure 50-8
Computerized tomography of a 28-year-old gentleman with a left posterolateral chest wall Ewing’s sarcoma. Of note is the destruction of the left ninth rib and the surrounding soft tissue mass.
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In the series by Burt and colleagues, 23% of patients had synchronous metastases at initial presentation. Of the patients who presented with only local disease, 71% developed distant metastases.
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Treatment is systemic chemotherapy, followed by resection or radiation therapy to the primary site.
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Doxorubicin, dactinomycin, cyclophosphamide, and vincristine in combination are the most frequently used chemotherapeutic agents.
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Using combined-modality therapy, 5-year event-free survival rates of 62% to 68% have been reported.
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In patients with distant metastatic disease, survival is extremely poor.
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Malignant Soft Tissue Tumors
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Malignant fibrous histiocytoma
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Most common primary chest wall neoplasm the thoracic surgeon is asked to evaluate.
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Usually occurs between the ages of 50 and 70 years, and is rare in childhood.
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Sixty-six occur in men.
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Presentation most commonly is a painless slowly enlarging mass.
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Grossly, it tends to be a lobulated mass, which spreads along fascial planes or between muscle fibers.
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Treatment is wide resection.
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It is unresponsive to chemotherapy and radiation therapy
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Five-year survival is approximately 38% ( Fig. 50-9 ).
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