Intracranial malignant triton tumor in a patient with neurofibromatosis type 1: Case report and review of the literature

Ross E. Smith, Meysam A. Kebriaei, Andrew P. Gard, Rodney D. Mccomb, Julia A. Bridge, Peter J. Lennarson

Research output: Contribution to journalArticle

6 Scopus citations


We report the fourth case of an intracranial malignant triton tumor not associated with a cranial nerve in a 26-year-old male with a clinical history of neurofibromatosis type 1. The patient was found unresponsive and displayed confusion, lethargy, hyperreflexia, and dysconjugate eye movements upon arrival at the emergency room. MRI revealed a large bifrontal mass. Biopsy demonstrated a high-grade spindle cell tumor with focal areas of rhabdomyoblasts that stained positive for desmin, myogenin, and muscle-specific actin. Electron microscopy showed skeletal muscle differentiation. Based on the clinical history of NF1 and the pathologic results, a diagnosis of malignant triton tumor was made. The differential diagnosis, immunohistochemistry, molecular genetics, and treatment of malignant triton tumor are reviewed.

Original languageEnglish (US)
Pages (from-to)149-154
Number of pages6
JournalBrain Tumor Pathology
Issue number2
StatePublished - Apr 2014



  • Frontal lobe
  • Intracranial
  • Malignant peripheral nerve sheath tumor
  • Malignant triton tumor
  • Neurofibromatosis

ASJC Scopus subject areas

  • Oncology
  • Clinical Neurology
  • Cancer Research

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