Radiation-induced malignant triton tumor associated with severe spinal cord compression

Abstract

Malignant triton tumor (MTT) is a variant of malignant peripheral nerve sheath tumors. The authors report a case of radiation-induced MTT in a patient with severe cervicothoracic cord compression and review the related literature. This 36-year-old man presented with pain and weakness in his left arm. His medical history was significant for a biopsy procedure involving the sampling of an aneurysmal bone cyst located at T1-3 near the left lung apex; this was performed 6 years prior to presentation and was followed by radiotherapy. Neurological examination demonstrated radicular findings involving the left C-8 and T-1 nerve roots. Neuroimaging studies revealed a large mass lesion extending from C-6 to T-2 along the vertebral column, invading the upper thoracic cavity and the adjacent lung apex, and infiltrating the paravertebral muscles. A subtotal resection was performed, but the tumor regrew extensively within a short time. It invaded the spinal canal and caused significant cord compression. The patient underwent surgery two more times for tumor debulking and to relieve progressive airway and spinal canal compromise. He eventually became quadriplegic, however, and died 13 months after diagnosis of MTT. This is the seventh case of radiation-induced MTT and the fifth of MTT with spinal canal involvement to be reported in the literature.