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http://dx.doi.org/10.12701/yujm.2019.00031

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

Jung, Chang Yeon (Department of Surgery, Yeungnam University College of Medicine)
Bae, Jung Min (Department of Surgery, Yeungnam University College of Medicine)
Choi, Joon Hyuk (Department of Pathology, Yeungnam University College of Medicine)
Jung, Ki Hoon (Department of Surgery, Dongguk University College of Medicine)
Publication Information
Journal of Yeungnam Medical Science / v.36, no.1, 2019 , pp. 63-66 More about this Journal
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.
Keywords
Nerve sheath neoplasm; Neurofibroma; Neurofibrosarcoma; Recurrent neurofibroma; Superficial MPNST;
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