Malignant Schwannoma of the Scalp in Type I Neurofibromatosis: Case Report

ABSTRACT

It has been reported that malignant transformation of neurofibroma occurs in about 5 to 10 per cent of type I neurofibromatosis patients. This 68 year old female patient presented with a huge fungating mass lesion in the right parietal scalp, which had recently grown rapidly. She had multiple neurofibromas of varying sizes on the entire trunk, extremities, and face. On the basis of family history and opthalomologic examination, type I neurofibromatosis was diagnosed. The mass was totally removed and the pathologic report revealed malignant transformation of the benign neurofibroma to malignant schwannoma. According to the literature, the incidence of head and neck sarcomas is very low, in particular malignant schwannoma occurring in the scalp is extremely rare. This case was, then, a rare event associated with type I neurofibromatosis.