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KMID : 1100320190360010063
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Yeungnam University Journal of Medicine
2019 Volume.36 No. 1 p.63 ~ p.66
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Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1
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Jung Chang-Yeon
Bae Jung-Min
Choi Joon-Hyuk
Jung Ki-Hoon
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Abstract
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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.
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KEYWORD
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Nerve sheath neoplasm, Neurofibroma, Neurofibrosarcoma, Recurrent neurofibroma, Superficial MPNST
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