Malignant Peripheral Nerve She ath Tumor – Two Case Reports

Czech version

Authors: V. Beneš lll ¹; F. Kramář ²; P. Hrabal ³; M. Kaiser ¹; P. Buchvald ¹
Authors‘ workplace: Neurochirurgické oddělení, Krajská nemocnice Liberec, 2Neurochirurgická klinika 1. LF UK, IPVZ a ÚVN Praha, 3Oddělení patologie ÚVN Praha ¹
Published in: Cesk Slov Neurol N 2009; 72/105(2): 163-167
Category: Case Report

Overview

Malignant peripheral nerve she ath tumor (MPNST) is a highly malignant tumor of the peripheral nervo us system, which is likely to reccur locally or metastatize. Its incidence is higher in pati ents with ne urofibromatosis type 1 (NF‑1). We present two case reports of pati ents with this rare tumor. First pati ent, 74- ye ar old woman, underwent ro utine chest exam which disclosed an expansive lesi on of the left chest cavity. Magnetic resonance imaging appe arance suggested a Th3 schwannoma. However, histopathological exam after parti al resecti on disclosed MPNST with proliferative potenti al of 25 %. The pati ent di ed six months later due to tumor progressi on. Second pati ent, 35- ye ar old woman with NF‑1 noticed enlargement of a known lump of the right calf. Histopathological exam after parti al resecti on disclosed ne urofibroma with signs of MPNST. Control magnetic resonance imaging reve aled massive tumor progressi on; the pati ent underwent limb amputati on. The pati ent has been in remissi on during 20 months follow‑up. Apart from these case reports the paper revi ews the literature as well as clinical and therape utical aspects of this tumor.

Key words:
malignant peripheral nerve she ath tumor – malignant schwannoma – ne urofibromatosis – soft tissue sarcoma

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