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Pediatr Neonatol. 2015 Feb;56(1):58-61. doi: 10.1016/j.pedneo.2013.01.013. Epub 2013 Mar 13.

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

Author information

1
Division of Hematology/Oncology, Department of Pediatrics, Chang Gung Children's Hospital, Chang Gung University, Taoyuan, Taiwan. Electronic address: jaing001@cgmh.org.tw.
2
Department of Plastic and Reconstructive Surgery, Chang Gung University, Taoyuan, Taiwan.
3
Department of Pathology, Chang Gung University, Taoyuan, Taiwan.
4
Division of Neurosurgery, Department of Surgery, Chang Gung University, Taoyuan, Taiwan.
5
Department of Radiation Oncology, Chang Gung Memorial Hospital, Chang Gung University, Taoyuan, Taiwan.
6
College of Medicine, Chang Gung University, Taoyuan, Taiwan.

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

PMID:
23597523
DOI:
10.1016/j.pedneo.2013.01.013
[Indexed for MEDLINE]
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