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Six decades ago, the epithelioid variant of malignant peripheral nerve sheath tumor (EMPNST) was described. This rare variant is different from the conventional MPNST. Superficial presentation of this tumor was believed associated with better behavior than deep presentation. To expand our understanding of this neural tumor, researchers now report on a review of 63EMPNSTs.
Males slightly predominated; median age was 44 years. Only one patient had neurofibromatosis 1 (NF1). The most frequent locations were the lower extremities (48%) and trunk (25%). Most cases (68%) were located in the dermis (5) and subcutaneous tissue (38), with an average size of 3 cm. The remainder were subfascial and visceral. Histologically, the tumors consisted of nest…