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The diagnosis of malignant peripheral nerve sheath tumor (MPNST) is made if a fibrosarcomatous-appearing tumor : 1) obviously arises from a peripheral nerve (as shown in this image); or 2) the tumor arises from a preexisting neurofibroma in NF1; or 3) the tumor has histologic features of a malignant Schwann cell tumor including hyper- and hypocellular areas, irregular wavy hyperchromatic nuclei with tapered ends, whorled structures, epithelioid appearance of perivascular tumor cells, and immunohistochemical or ultrastructural evidence of Schwann cell differentiation.

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