Fritz J. Baumgartner
The tumors of the chest wall fall into several classifications. They may be of bony or soft tissue origin. They may be benign or malignant and those that are malignant may be primary or metastatic. They may be lesions that invade the chest wall from the lung, pleura, mediastinum or breast.
Benign rib tumors include osteochondroma, chondroma, fibrous dysplasia and histiocytosis X. Malignant rib tumors include multiple myeloma, osteosarcoma, chondrosarcoma and Ewing's sarcoma. Osteochondroma is the most common benign bone neoplasm, constituting 50% of benign rib tumors. Multiple myelomas are the most common primary malignant rib neoplasm accounting for one-third of all malignant rib tumors, with chondrosarcoma being a close second. Benign soft tissue chest tumors include fibromas, lipomas, neurogenic tumors, hemangiomas and desmoid tumors.
Malignant fibrous histiocytoma (MFH) is the most common malignant soft tissue tumor. The tumor characteristically occurs in late adulthood, and is rare in childhood. The disease often is a painless, slowly growing mass and be more common after chest wall irradiation. Malignant fibrous histiocytoma can spread widely along fascial planes or between muscle fibers accounting for a high recurrence rate after resection. MFH is unresponsive to radiation and chemotherapy and should be widely resected. Five year survival overall is approximately 40%.
Rhabdomyosarcoma is the second most common chest wall soft tissue malignancy. Wide resection followed by radiation and chemotherapy results in 5 year survival of about 70%. Liposarcoma and leiyomyosarcoma are other forms of soft tissue tumors. With regard to osteochondroma, the onset of pain in a previously asymptomatic tumor may indicate malignant degeneration. The neoplasm usually begins in childhood and continues to grow until skeletal maturity. Osteo-chondromas arise from the metastasis.
Chondromas constitute 15% of benign neoplasms of the rib cage. The differentiation between chondroma and chondrosarcoma is impossible on a clinical and x-ray findings. Also, microscopically, the chondroma tumor and a low grade chondrosarcoma may look identical; therefore, all chondromas must be considered malignant and should be treated by wide excision. Although this resection may appear to be excessive, the risk is negligible and may be life-saving.
Fibrous dysplasia presents, in most cases, as solitary lesions, however when multiple lesions are encountered (polyostotic fibrous dysplasia), then Albright syndrome may be present (multiple bone cyst, skin pigmentation and precocious sexual maturity in girls).
Histiocytosis X is a disease involving the reticuloendothelial system and includes eosinophilic granuloma, Letterer-Siwe disease, and Hans-Schuller-Christian disease. Radiation therapy is frequently given. Steroid and chemotherapy may also be needed.
Myelomas involving the chest wall usually occur as a manifestation of systemic multiple myeloma. Solitary myeloma involving the rib is secondary only to solitary vertebral involvement. Multiple myeloma is rare under the age of 30. Most of the patients have abnormal electrophoresis and half have hypercalcemia and Bence-Jones protein in the urine.
Chondrosarcoma usually arises in the costochondral junction or the sternum. It is relatively uncommon under the age of 20. It most commonly presents as a slowly growing mass which has been painful for several months. All tumors arising in the costal cartilages should be considered malignant and should be treated by wide resection. The etiology of chondrosarcoma is unknown and may include malignant degeneration of chondromas, but may also be associated with trauma. Definitive diagnosis of chondrosarcoma can only be made pathologically, however even this is sometimes difficult because most chondrosarcomas are well differentiated. This is why wide resection of chondromas is necessary, since these may in fact be chondrosarcomas. The objective of the first operation should be resection wide enough to prevent local recurrence. This involves resection of a 4 cm margin of normal tissue on all sides. This results in cure in nearly all patients with a 10 year survival of 97%.
Ewing's sarcoma results in onionskin appearance of the surface of the bone on x-ray caused by elevation of the periosteum with subperiosteal new bone formation of multiple layers. Early spread to the lungs and other bones is common and occurs in about half of patients. Ewing's sarcoma is radiosensitive, so this modality is the treatment of choice. Five year survival is 50%. It may appear to be predominantly subcutaneous (Fig. 15.1).
Osteosarcoma is more malignant than chondrosarcoma and has a less favorable prognosis. Osteosarcoma usually occurs in teenagers and young adults who present with a rapidly enlarging, painful tumor. Alkaline phosphatase levels are frequently elevated. Calcification occurs at a right angle to the cortex producing a sunburst appearance on x-ray. Osteosarcoma treatment consists of wide resection of the tumor including the entire bone, rib or sternum and adjacent soft tissue.
Chest Wall Tumors
The role of radiation and chemotherapy is controversial. The prognosis is poor with 5 year survival rates of 20%.
The management of suspected chest wall tumors include a careful history and physical examination followed by plain x-ray and CT scan. Tumors clinically suspected of being a primary neoplasm should be diagnosed by excisional rather than incisional or needle biopsy. However, if there is a history of primary neoplasm elsewhere and the chest wall mass may be a metastasis, then needle or incisional biopsy to establish diagnosis is acceptable. If a lesion is diagnosed by excisional biopsy as a malignant neoplasm, then further excision is indicated to include all of the involved bone and a 4 cm margin of normal tissue on all sides. This includes removal of the involved ribs and a rib above and below the neoplasm. For tumors of the sternum and manubrium, excision of the entire involved bone and corresponding costal arches bilaterally is indicated.
Metastases after they are diagnosed by biopsy do not require further surgical treatment. Radiation is the treatment of choice. Desmoid tumors tend to recur if inadequately excised and therefore should be treated with wide resection similar to primary malignant chest wall tumors. Bony defects should be reconstructed if large. Defects less than 5 cm in greatest diameter anywhere on the thorax are usually not necessarily reconstructed. Posterior defects less than 10 cm also do not require reconstruction because the scapula provides support. Larger defects elsewhere should be reconstructed. This can be performed with Prolene mesh or 2 mm Gortex soft tissue patch. Relative contraindications to patch implantation are an infected or necrotic field. Muscle flaps may be necessary to reconstruct the chest wall. This can be done with latissimus dorsi, pectoralis major, serratus anterior, or rectus abdominus in descending order of preference.
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