Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1
- Author:
Chang Yeon JUNG
1
;
Jung Min BAE
;
Joon Hyuk CHOI
;
Ki Hoon JUNG
Author Information
- Publication Type:Case Report
- Keywords: Nerve sheath neoplasm; Neurofibroma; Neurofibrosarcoma; Recurrent neurofibroma; Superficial MPNST
- MeSH: Abdominal Wall; Humans; Nerve Sheath Neoplasms; Neurilemmoma; Neurofibroma; Neurofibromatoses; Neurofibromatosis 1; Neurofibrosarcoma; Peripheral Nerves; Prognosis; Sarcoma
- From:Yeungnam University Journal of Medicine 2019;36(1):63-66
- CountryRepublic of Korea
- Language:English
- Abstract: Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.
