Malignant Triton Tumor (Malignant Peripheral Nerve Sheath Tumor With Rhabdomyoblastic Differentiation) Occurring in a Vascularized Free Flap Reconstruction Graft.

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare form of sarcoma arising from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT) is a subtype of MPNST with a component of malignant rhabdomyoblasts in addition to malignant Schwann cells. MPNST and MTT are both aggressive malignancies that most commonly arise from large deep neurofibromas in patients with neurofibromatosis type 1 (NF-1). However, sporadic non-NF-1 cases of MTT have also been reported in the literature. We present a case of a 50-year-old African American male with no stigmata of NF-1 who developed a large mass in a free flap on the right calf. The free flap had been placed by plastic surgery 15 years previously following open right tibial and fibular fractures associated with a large soft tissue defect. Biopsy of the enlarging mass, followed by wide surgical excision, confirmed the pathology to be MTT. Although the development of a high-grade soft tissue sarcoma has been previously reported as a late complication of radiation therapy following free flap reconstructions, we believe this is the first reported case of sarcomatous transformation arising in a vascularized, free muscle transfer.