Malignant Triton Tumor of the Cervical Spine: Report of One Case and Review of the Literature

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-ye...

Full description

Saved in:
Bibliographic Details
Main Authors: Tang-Her Jaing, Chwei-Chin Chuang, Shih-Ming Jung, Chieh-Tsai Wu, Chen-Kan Tseng, Chang-Sheng Chen
Format: Article
Language:English
Published: Elsevier 2015-02-01
Series:Pediatrics and Neonatology
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S1875957213000144
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:1875-9572