Malignant triton tumor of the cervical spine: report of one case and review of the literature

Abstract

Malignant triton tumor (MTT) is a highly aggressive malignant neoplasm, classified as a variant of malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. MTT is rarely reported in children, and its true prevalence may be underestimated. We herein report such a case in an 8-year-old boy who presented with a mass over the trapezius muscle. He was previously diagnosed with neurofibromatosis in the same area. Four years later, a follow-up magnetic resonance imaging revealed an intradural tumor recurrence at the level of C1-C7. An immunohistochemical test result was positive for S-100 protein and desmin, which confirmed the diagnosis. The patient outcome was fatal despite multimodal therapy. The possibility of this rare but devastating tumor must always be considered when patients present with new compressive spinal symptoms.

Keywords: malignant peripheral nerve sheath tumor; malignant triton tumor; neurofibromatosis.