Malignant chest wall tumors in children and young adults

PURPOSE: Primary chest wall malignancies, which occur infrequently in children, can pose complex technical challenges to the surgeon. This study was undertaken to evaluate the pathology, treatment approaches, role of surgical resection and reconstruction, and outcomes of patients with these tumors. METHODS: This is a retrospective review of all patients with malignant primary chest wall tumors treated at our institution between February 1983 and July 1998. RESULTS: Nineteen cases were identified: malignant small round cell type (MSRCT, also called Ewing's sarcoma, primitive neuroectodermal tumor PNET], and Askin's tumor; n = 8), rhabdomyosarcoma (RMS; n = 6), and other tumors (n = 5). Three patients underwent "upfront" complete resections. Sixteen patients underwent initial biopsy, followed by chemo- or radiotherapy. Nine of these 16 survived to undergo delayed chest wall resections. Six of the 12 "resected" patients required en bloc resection of adjacent muscles or organs; 7 required complex chest wall reconstruction. Eight of 19 patients (42%) have survived (median follow-up of survivors, 4 years), all with no evidence of disease; the remaining 11 patients died of progressive disease. Local invasion did not alter chance of survival. Two of the 10 patients with metastases at diagnosis (20%) survived. Six of the 9 patients (67%) with localized disease survived. All five patients with tumor types other than MSRCT or RMS, metastatic or not at diagnosis, are alive with no evidence of disease. There were no local recurrences. CONCLUSION: Surgical resection, with en bloc removal of involved structures and chest wall reconstruction, provides excellent local control of malignant chest wall tumors.