Ulcerated malignant spindle-cell neoplasm of the finger: Malignant peripheral nerve sheath tumor or desmoplastic malignant melanoma?
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Malignant peripheral nerve sheath tumors (MPNSTs) usually occur in the deep soft tissues in patients with neurofibromatosis type 1 (NF-1) in association with or arising from a peripheral nerve, but MPNSTs may also occur in patients without evidence of NF-1. MPNSTs used to be referred to as neurofibrosarcomas, neurogenic sarcomas, malignant schwannomas, and ‘‘Triton tumors’’ when heterologous mesenchymal elements such as rhabdomyosarcoma, leiomyosarcoma, chondrosarcoma, or osteosarcoma were present, but MPNST is now the preferred term. MPNSTs may also arise in the skin, and these neoplasms are referred to as cutaneous or superficial MPNSTs (SMPNSTs). MPNSTs, as a group, are rare, and SMPNSTs are even rarer.
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