Malignant triton tumor: Grand Round presentation of a rare aggressive case thoracolumbar spine tumor.

Abstract

We report a rare and aggressive case of malignant triton tumor (MTT) at the thoracolumbar junction with foraminal extension mistreated as schwannoma. A 70-year-old man with a 2-year history of lower back pain and left L4 radiculopathy with no history of neurofibromatosis. Pre-operative MRI suggested a typical schwannoma. Upon complete marginal resection, histological findings revealed a MTT. The patient presented with a local and regional recurrence and died 10 months after surgery. MTTs are a subgroup of malignant peripheral nerve sheath tumors, which develop from Schwann cells of peripheral nerves or within existing neurofibromas, and display rhabdomyoblastic differentiation. Based on the Grand Round case and relevant literature, we present a case of a highly aggressive and fast-growing tumor with a very high local and distant recurrence. There is no consensus treatment plan available and patients usually die shortly after diagnosis.