Primary Chest Wall Tumors

doi:10.1016/j.thorsurg.2010.07.003

Thoracic Surgery Clinics

Volume 20, Issue 4, November 2010, Pages 495-507

https://doi.org/10.1016/j.thorsurg.2010.07.003 Get rights and content

Introduction

Patients with chest wall tumors present diagnostic and therapeutic challenges. The differential diagnosis of these tumors is broad, because they can represent a heterogeneous spectrum of diseases from primary benign or malignant tumors to metastases; local extension of adjacent tumors of the lung, mediastinum, pleura or breast; non-neoplastic infectious or inflammatory conditions; or even local manifestations of systemic disease. Primary chest wall tumors are best classified according to their tissue of origin, bone or soft tissue, and further subclassified according to whether or not they are benign or malignant. Most of these tumors are uncommon, with information garnered from individual case reports or institutional case series.

Chest wall tumors are more commonly either metastases or local invasion of an underlying adjacent tumor. Primary chest wall tumors account for only 0.04% of all new cancers diagnosed and 5% of all thoracic neoplasms.¹ The list of potential tumors is broad (Table 1), with nomenclature that is frequently overlapping and sometimes contradictory in the literature. Primary benign lesions of the chest wall can behave in a latent, active, or aggressive manner. Approximately 60% of primary chest wall tumors are malignant. Although primary chest wall tumors are diagnosed in every age group, they are more likely malignant in the extremes of age: in the young and the elderly. Certain tumors present predominantly in one age group. For example, Ewing sarcoma is more common in children and young adults, primitive neuroectodermal tumor (PNET) in patients in their 20s, and chondrosarcoma in middle adult life and solitary plasmacytoma occurs more frequently in older adults. Of malignant tumors, chondrosarcoma and lymphoma are most prominent in adults,² whereas in children, Ewing sarcoma and rhabdomyosarcoma are the most common tumors.³

Section snippets

Diagnosis

Patients often present with a palpable enlarging mass. Less commonly, asymptomatic patients are diagnosed due to an incidental finding on imaging as part of screening or for investigation of an unrelated condition. Soft tissue masses are often painless, whereas bony lesions, both benign and malignant, are typically painful due to growth and periosteal damage. Symptoms develop as the tumor grows and can be associated with local invasion of adjacent structures. Paresthesias and weakness may be

Primary Tumors of the Rib

Primary tumors of the rib comprise only 5% to 7% of all primary bone neoplasms9, 10 but make up 50% of bony malignant tumors and the majority of benign bony tumors of the chest wall. Tumors of the rib are derived from bone, cartilage, bone marrow, vascular, or neural structures. Of the benign lesions, fibrous dysplasia and chondroma are the most common, ⁹ whereas chondrosarcoma and osteosarcoma are the most common malignant rib lesions.¹¹ Ewing sarcoma is the most common malignant rib tumor in

Benign Bone Tumors

The most common benign bony tumors of the chest wall include fibrous dysplasia (30%–50%), osteochondroma (30%–50%), chondroma (10%–25%), aneurysmal bone cyst (10%–25%), and eosinophilic granuloma,¹ with many other rarer tumors reported in the literature (see Table 1).

Benign soft tissue tumors

The differential of soft tissue abnormalities is vast and, in addition to primary tumors, should encompass local presentations of systemic diseases as well as infectious causes, such as tuberculosis and actinomycosis. In terms of primary benign soft tissue tumors (see Table 1), the most common diagnoses include lipomas, fibromas, hemangiomas, and giant cell tumors.³² Less common diagnoses include lipoblastomas and mesenchymal hamartomas.

Malignant soft tissue tumors

More common than malignant tumors of the bony thorax, most malignant soft tissue tumors of the chest wall are sarcomas, including malignant fibrous histiocytomas, liposarcomas, and fibrosarcomas. They are often asymptomatic and more commonly found on the anterior chest wall. Most sarcomas have a poor prognosis, with survival influenced by histology, tumor grade, diameter, and location. Reported overall 5-year survival is still only 50% to 66%, although low-grade well-differentiated tumors

Surgical treatment of chest wall tumors

The indication for surgery is based on evaluation of the tumor histology, location, degree of local invasion, and presence of metastases. Localization of small tumors, or for those with significant response to neoadjuvant treatment, may be difficult. Video-assisted thoracoscopic surgery may facilitate tumor localization if the tumor is visible from the pleural surface. Entering the pleura one or two ribs above or below the lesion may allow palpation of the defect. If the lesion is too small to

Summary

The differential diagnosis of patients presenting with a lesion on the chest wall must take into account local anatomy as well as patient age, gender, previous history of malignancy, and exposure to radiation. In addition to benign or malignant primary chest wall tumors, diagnostic possibilities include local presentations of systemic disease, infection, metastasis, or extension of underlying malignancies. With particular attention to imaging characteristics of the lesion, a diagnosis can often

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