Malignant Peripheral Nerve Sheath Tumors
Malignant peripheral nerve sheath tumors (MPNSTs) (ICD-O 9540/3) are rare soft-tissue sarcomas that arise predominantly from Schwann cells. A malignant spindle-cell tumor with histological, immunohistochemical, and ultrastructural features of Schwann cell differentiation, they usually arise in the setting of neurofibromatosis 1 (NF1) or may be seen sporadically in children.
KeywordsMalignant neurofibroma Malignant schwannoma Peripheral nerve sheath tumor MPNST Schwann cell
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