CHEST-WALL RHABDOMYOSARCOMA

BACKGROUND. Rhabdomyosarcoma is the most common soft tissue sarcoma in the pediatric age group. The primary tumor site is an important progn ostic determinant. Axial lesions are associated with decreased surviva l and provide a clinical challenge. METHODS. A retrospective analysis of the authors' institutional experience between 1972 and 1996 was per formed. Patients were from a data base of 302 consecutive cases. RESUL TS, Fifteen consecutive patients with chest wall rhabdomyosarcoma were identified. The median age was 16 years (range, 6 months-25 years). M edian followup was 6.6 years (range, 10 months-18.5 years). Nine patie nts presented with a mass, six with pain, two with respiratory distres s, and one with ulnar neuropathy. The median lesion size was 7 cm (ran ge, 3-16 cm). A surgical procedure was the initial therapy for 13 of 1 5 patients. Fourteen patients received radiation therapy with a median dose of 4400 cGy. All but one were included in institutional-based tr ials using multiagent chemotherapy. At last follow-up, 10 patients wer e alive and disease free, with a median survival of 123 months (range, 51-221 months). Seven of ten survivors underwent a complete resection as their initial therapy. There was no surgical mortality, and only t wo patients had treatment-related complications. Of the five patients who died, two underwent complete resection as their initial therapy. A ll five patients had invasive tumors. Four were > 10 cm, 3 were of alv eolar subtype, and 2 were embryonal. CONCLUSIONS, Complete resection o f chest wail rhabdomyosarcoma is recommended. However, survival is pos sible for patients with microscopically positive surgical margins with the addition of chemotherapy and radiation. (C) 1997 American Cancer Society.