Abstract: Neurofibrosarcoma is a rare malignant nerve sheath tumor with a lot of histological entities. In 70% of the cases, it is associated with neurofibromatosis type 1 (NF1). We report the case of a 44-year-old female patient without neurofibromatosis, who was diagnosed with a neurofibrosarcoma of the median nerve of the left hand. The location in the hand of neurofibrosarcoma is exceptional because of its histological nature, especially outside the context of neurofibromatosis. The treatment involves surgery, chemotherapy, and radiotherapy with a high risk of local recurrence.
We provide through this case report a review of the literature and discuss the diagnosis, histological features, hypothetical etiologies, different therapeutic options and prognosis of this tumor.
