Neurofibrosarcoma of thigh arising in a case of neurofibromatosis a case report

ABSTRACT

This is a report of 14 year old boy who had neurofibromatosis Type-I, with plexiform neurofibromas, multiple cafe-au-lait spots, papillomas throughout the body and a large diffuse lesion of left thigh. The latter had completely disabled him. Investigations revealed this lesion to be a case of neurofibrosarcoma, which is a malignant peripheral nerve sheath tumor. Details of management are discussed in this report. A brief review of literature is also presented