Malignant triton tumors of the spine

Abstract

✓Malignant triton tumors (MTTs) are malignant peripheral nerve sheath tumors with rhabdomyosarcomatous differentiation. Malignant triton tumors affecting the spine are rare but present special challenges to the neurosurgeon. The authors report on 2 new cases of spinal MTTs, and analyze the 8 previously reported cases found via database search.The patients include a 15-year-old girl with thoracic MTT, and a 24-year-old man with lumbosacral MTT; both patients underwent radical resection with spinal stabilization. When these cases were combined with the literature results there was a male/female ratio of 6:4, and an age range of 15–67 years (median 37.5). Nine patients presented with symptoms related to the spinal cord, cauda equina, or nerve root compression. Four patients had undergone previous radiotherapy, and 2 had neurofibromatosis Type 1. Five cases of MTT were lumbosacral, 4 were thoracic, and 1 was cervical. Seven patients had intradural extension of tumor. All patients underwent some form of surgery, with 8 having total macroscopic excision. However, 6 patients required subsequent operations. In the 8 patients for which survival data was available, median time from diagnosis to death was 12 months (range 3–16).Malignant triton tumors are rare but should be included in the differential diagnosis of spinal tumors, particularly in patients who have undergone previous radiotherapy or who have neurofibromatosis. The authors suggest roles for radical surgery and multidisciplinary management.