Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1

• Main Authors: Chang Yeon Jung, Jung Min Bae, Joon Hyuk Choi, Ki Hoon Jung
• Format: Article
• Language: English
• Published: Yeungnam University College of Medicine 2019-01-01
• Series: Yeungnam University Journal of Medicine
• Subjects: Nerve sheath neoplasm; Neurofibroma; Neurofibrosarcoma; Recurrent neurofibroma; Superficial MPNST
• Online Access: http://yujm.yu.ac.kr/upload/pdf/yujm-2019-00031.pdf
• ISSN: 2384-0293

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.