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

Abstract

[InlineMediaObject not available: see fulltext.]Introduction: We report a rare and aggressive case of malignant triton tumor (MTT) at the thoracolumbar junction with foraminal extension mistreated as schwannoma. Materials and methods: A 70-year-old man with a 2-year history of lower back pain and left L4 radiculopathy with no history of neurofibromatosis. Results: 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. Conclusion: 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.