Chest wall tumors

Abstract

Chest wall tumors in children include a whole range of primary neoplasm arising from different components of the chest wall or secondary tumors invading the chest wall and originating from the adjacent structures such as breast, pleura, mediastinum, and lung. In addition, metastatic tumors also present diagnostic and therapeutic challenges to a pediatric thoracic surgeon. Chest wall tumors both benign and malignant are rare in children. In many of these tumors, complete surgical resection is frequently the only modality of oncological management. Furthermore, reconstruction of the wide chest wall defects poses a surgical challenge in a child. Primary tumors of the chest wall arising from bony and soft tissue skeleton account for less than 2% of the primary body tumors in children.1 Soft tissue tumors of the chest wall are common compared with skeletal bony tumors. Overall reported incidence of solid childhood soft tissue tumors is 1.8%, and majority of these tumors are mesenchymal in origin and can have malignant behavior.2 Most common primary malignant chest wall tumor reported in children is primitive neuroectodermal tumor (PNET),3 followed by rhabdomyosarcoma (RMS) and other rarer soft tissue sarcoma namely liposarcoma and fibrosarcoma. Various nomenclature in the literature has been used describing essentially same kind of soft tissue sarcoma; Ewing's sarcoma, primitive neuroectodermal and Askin's tumor4 due to lack of distinction among these lesions with regards to their neuroectodermal differentiation. Recently, pathologists' have grouped them as a single entity as malignant small round cell tumors (MSRCT).5 This tumor poses significant diagnostic challenge and requires number of immunohistochemical stains to differentiate it from other tumors.