David H. Harpole, Jr., MD
Duke University Medical Center, Durham, North Carolina
Chest wall tumors are an unusual problem, however, it is important to understand these lesions to provide adequate therapy. The useful classification of chest wall tumors includes primary tumors of the bone (ribs, sternum and vertebrae), metastatic bone lesions, tumors that invade the chest wall (lung pleura, mediastinum and breast), soft tissue chest wall tumors and pseudotumors of the bone and soft tissues. Usual therapy involves some type of excision.
Presentation
Most commonly, patients present with an asymptomatic lesion which is observed on an elective chest radiograph. Inward growth without surface manifestation can allow these masses to reach a relatively large size. The most common symptom is chest wall pain (burning and pleuritic), followed by dyspnea due to pulmonary displacement. Patients often describe an association with trauma (unrelated). Large bone or soft tissue lesions can present with a non-tender palpable mass. If the mass if pulsatile, it is usually metastatic renal cell or thyroid carcinoma. The presence of a relatively rapid growing, painful, anteriorly-located lesion suggests malignancy.
Diagnosis
Metastatic tumors are much more common than primary chest wall lesions. Therefore, a metastatic survey should be undertaken to identify the tumor primary and other metastatic deposits. Radiological examinations of the chest include chest/abdominal computed tomography, radionuclide bone scan and magnetic resonance imaging.
Controversy exists about the need for a tissue diagnosis prior to definitive therapy. Biopsy can be undertaken if the radiological examinations are unable to suggest a diagnosis. Small lesions should have an excisional biopsy, while larger ones may require an incisional biopsy. Care must be taken to create an incision which does not violate surrounding tissue planes. The biopsy site must be excised at the time of the definite resection, reducing the likelihood of localized tumor dissemination.
Tumors of the Bone
(1) Rib Tumors
Primary Rib Tumors:
The incidence of primary rib tumors is highest between ages 30 and 60 years. Presentation is at an average 2 to 3 years after the initiation of symptoms. Forty percent are malignant (chondrosarcoma>plasmacytoma). The common benign tumors are chrondroma>ostecolastoma (benign giant cell tumor), while the most common lesion in children is a osteochondroma. Primary rib tumors should be resected with 10 to 15 cm longitudinal margin at each end, include at least 1 normal rib above and below the lesion and include a complete resection of the overlying soft tissue.
Metastatic Rib Tumors:
The incidence of metastatic rib tumors is greatest after age 60 years. Symptom are usually serve, with presentation less than 6 months after initiation. Often the lesions are multiple and a pathologic fracture is present on radiograph. The primary cancers are (common) lung, breast, and prostate>testicular, gastrointestinal, gynecological, parathyroid, thyroid and limb sarcoma. If a single lesion is present, a limited resection for symptomatic control may be recommended.
(2) Sternal Tumors
The Most common tumors of the sternum are metastatic (thyroid, kidney and breast>lung, gastric and testicular. Primary tumors are usually malignant and are extremely rare (sarcoma>plasmacytoma). Resection should include the entire sternum to manubrium, costal cartilages and overlying soft tissue. A manubrial tumor resection should include the medial 1/3 of the clavicles, proximal sternum and overlying soft tissue.
Tumors that Invade the Chest Wall
(1) Lung Cancer
Primary chest wall-invasive lung cancer is usually non-small cell lung cancer.
Superior Sulcus Tumors:
Lesions in the superior sulcus (Pancoast) can present with localized pain, brachial plexus symptoms and sympathetic nerve dysfunction (Horner's). Primary resection after radiation therapy provides an 80% local control rate and a 30 to 40% overall survival. Most recurrence is at a distant site. Patients with T3NO (stage IIB) lesions may have a 50% 5-year survival. A prospective, multi-institutional trial of velban+cisplatine+external beam irradiation is ongoing in an attempt to decrease distant relapse.
Lateral or Posterior Chest Wall Tumors:
Lateral or posterior chest wall-invasive lung cancers often present with localized pain. Rib destruction can be seen of chest x-ray or computed tomography. No standard therapy exists, with most patients undergoing a primary resection, followed by external beam irradiation for positive margins or positive lump nodes. A 50% 5-year survival can be reached for patients with T3NO tumors (stage IIB).
Medial Chest Wall Tumors:
These tumors are often identified late in the course of the cancer, and usually are inoperable due to direct involvement of the mediastinal structures.
(2) Breast Cancer
Primary breast cancer which invades the chest wall is unusual today. However, locally aggressive, recurrent breast cancer after a modified radical mastectomy of lumpectomy and radiation is observed in large breast cancer centers. If there is no evident of distant metastases, a chest wall resection (with underlying ribs if involved) can significantly palliate many patients.
(3) Mesothelioma
Malignant Pleural Mesothelioma is an unusual, asbestos-related tumor with approximately 3500 cases annually. Once the tumor has invaded through the sub-pleural fibrous tissue and into the overlying chest wall, it is stage III (inoperable).
Soft Tissue Tumors of the Chest Wall
The chest wall is an unusual location for a soft tissue tumor, but these make up approximately 40% of the primary malignant chest wall tumors. The most common are posterior mediastinal sarcoma which directly invades the chest wall (neurogenic tumors). These lesions are often dumbell-shaped, having a small intra-thecal component. Therefore, it is wise to obtain a magnetic resonance scan of the spinal canal prior to resection. Benign desmoid tumors can slowly grow in the chest wall, requiring resection. Although these lesions are not malignant, they often recur after resection. True soft tissue sarcomas can occur in any of the connective tissue of the chest wall. The most common are malignant fibrous histiocytomas or rhabdomyosarcomas. These tumors can usually be resected en-bloc, without destruction of the underlying, uninvolved structures. However, if the ribs are incorporated in the tumor mass, a full-thickness chest wall resection may be necessary.
Pseudotumors of the Ribs and Chest Wall
These lesions are most common in patients under 20 years of age. The mass is usually asymptomatic or presents with mild discomfort of an average one year's duration. The most common is Fibrous dysplasia (osteitis fibrosa) is a locally expanded rib with the classic "soap bubble" appearance on radiograph. The elderly or immunocompromised host can present with a rapidly enlarging, tender, inflammatory lesion [actinomycosis "sulfur granules" or empyema necessitatis (bacterial or my cobacterial)]. Finally, a giant aortic aneurysm can rarely present as a pulsating chest wall mass.
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