Neurofibrosarcomas of the upper extremity.

Abstract

A retrospective review of all malignant hand tumors seen at the University of Michigan from 1950 to 1987 demonstrated six biopsy-proven cases of neurofibrosarcoma involving the upper extremity. There were four male and two female patients; average age at presentation was 37 years and ages ranged from 15 to 63 years. All patients had a history of von Recklinghausen's disease. Three patients were seen initially with painful enlarging masses, one with a painful mass with a neurological deficit, and two with painless enlarging masses. Three patients had radical excisions requiring upper extremity or forequarter amputation, and three patients received limited or no surgical treatment. Five patients died of metastases an average of 3 years after diagnosis. One patient remains alive 18 years after upper extremity amputation despite an early local recurrence. Early diagnosis and radical surgical excision offer the best chance of long-term survival from this highly lethal cancer.