(A) A positron emission tomography/computed tomography study in a patient with neurofibromatosis (NF) type 1-associated malignant peripheral nerve sheath tumor (MPNST) is depicted. The MPNST is located in the right distal thigh (maximum standard uptake value [SUVmax], 14.9) and arises from a benign neurofibroma. A neurofibroma with lower F18-fluorodeoxyglucose uptake (SUVmax, 2.3) is located in the left medial thigh. (B–D) The histologic findings of the patient’s right thigh lesion are depicted. (B) A region of benign neurofibroma (original magnification, ×20) shows the usual low cellularity and slender spindled cells with interspersed pink collagen fibers. Note that no mitoses or necrosis is present. (C) A low-power view (original magnification, ×2) shows transition from neurofibroma (lower left corner) to high-grade MPNST (upper right corner). Note the increase in cellularity from the benign to the malignant area. (D) A high-power view (original magnification, ×20) of the high-grade MPNST is shown. Note the marked increase in cellularity, obvious mitoses, and nuclear pleomorphism. Extensive necrosis (not pictured here) was also present.