[Sarcomatous degeneration in Recklinghausen's neurofibromatosis]

During a 6-year period only a single case of sarcomatous transformation in neurofibromatosis (v. Recklinghausen's disease) was found among 1561 autopsies. The clinical course and histopathological changes of this rare case are described. The incidence of sarcomatous transformation seems to be less than 3%, in contrast to some data reported in the literature. These fibrosarcomas of the nerve sheaths usually originate in subfascial nodes and in other clinically poorly accessible locations. Some cases in the literature indicate genetic factors to be responsible for the sarcomatous transformation. Therefore, patients with such a family history should be followed closely.