title: Intracranial malignant triton tumor in a patient with neurofibromatosis type 1: case report and review of the literature.
abstract: 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.
authors: Division of Neurosurgery, Department of Surgery, University of Nebraska Medical Center, 11364 Gold St, Omaha, NE, 68144, USA, rsmithe@unmc.edu.
authors: Ross E
authors: RE
authors: Smith
authors: Meysam A
authors: MA
authors: Kebriaei
authors: Andrew P
authors: AP
authors: Gard
authors: Rodney D
authors: RD
authors: McComb
authors: Julia A
authors: JA
authors: Bridge
authors: Peter J
authors: PJ
authors: Lennarson
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology / Q000628:therapy; D003131:Combined Modality Therapy; D003933:Diagnosis; D017809:Fatal Outcome; D005625:Frontal Lobe; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D025202:Molecular Diagnostic Techniques; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000473:pathology / Q000628:therapy; D009456:Neurofibromatosis 1 / Q000150:complications*
chemical_list: D014408:Biomarkers, Tumor