Malignant Peripheral Nerve Sheath Tumor of the Neck: Transformation From a Recurrent Neurofibroma in a Patient Without Neurofibromatosis

Chen, Han-Ju; Chen, Huan-Sen; Chang, Yen‐Liang; Wu, Yi-Yiing

doi:10.1016/s1016-3190(10)60071-7

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“…In addition, MPNST of the skin or subcutaneous layer is very rare [8]. NF1 MPNSTs account for about half of all MPNSTs; the remainder are nearly all sMPNSTs [1,4]. The median age of patients with sMPNSTs is between 30 and 60 years [2]; the size of these tumors is smaller, and the stage shows less progression than NF-MPNSTs [1].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

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

Jung¹,

Bae²,

Choi³

et al. 2019

Yeungnam Univ J Med

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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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Section: Discussionmentioning

confidence: 99%

“…Superficial MPNST in the cutaneous or subcutaneous tissue is very rarely found without NF1. Moreover, recurrent neurofibroma that transforms to MPNST is extremely rare, with only three cases reported by Chen et al [4].…”

Section: Introductionmentioning

confidence: 99%