Different Pathology of Peripheral Nerve Sheath Tumor in Neurofibromatosis Type 1: 3 Cases

Abstract

Neurofibromatosis type 1 (NF1) is an autosomal-dominant genetic disease that predisposes affected individuals to tumors. Neurofibroma and malignant peripheral nerve sheath tumor (MPNST) are examples of PNSTs that occur either sporadically or as part of hereditary neurocutaneous diseases such as NF1. We treated three patients with NF1 who presented with different PNSTs. All patients underwent surgical resection, and pathologic findings indicated neurofibroma, atypical neurofibroma, and MPNST, respectively. We managed each case based on its pathology. The patient with MPNST died after chemoradiotherapy; the other patients did not experience recurrence for several months.