Expert’s comment concerning Grand Rounds case entitled "Malignant triton tumor: Grand Round presentation of a rare aggressive case thoracolumbar spine tumor" by S. Ghailane et al. (Eur Spine J; 2017: DOI 10.1007/s00586-017-5277-2)

Abstract

The authors report a case of malignant triton tumor of the spine, presenting at the thoracolumbar of a 70 year old gentleman, with back pain and left leg pain of two years duration. Unfortunately, 3 months after complete marginal resection, the patient developed local recurrence and seeding to the cauda equina, and died 10 months after diagnosis. Malignant triton tumor is a rare variant of malignant peripheral nerve sheath tumor that has rhabdomyoblastic differentiation [1]. The tumor originates from neural crest cells, and is a high grade sarcomatous lesion. Median age at presentation is 29 years, with male to female ratio 1.5:1. Overall survival at 18 months is around 45%, but adverse factors include incomplete resection, spinal involvement, size greater than 2cm, or association with neurofibromatosis. Accepted treatment is complete excision and post-operative radiotherapy, and sometimes chemotherapy. However, as in this case, resection of extensive spinal tumors is rarely complete.