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Clinical diagnostic and radiographic features of recurrent intracranial malignant triton tumor in an adult: illustrative case
Journal article   Open access

Clinical diagnostic and radiographic features of recurrent intracranial malignant triton tumor in an adult: illustrative case

Ajmain Chowdhury, Alex Belzer, Nahom Teferi, Hend Al-Kaylani, Juan Vivanco-Suarez, Assim Saad Eddin, Kathryn Eschbacher, Mohammed Milhem and Patrick Hitchon
Journal of Neurosurgery: Case Lessons, Vol.6(8), CASE23338
08/21/2023
DOI: 10.3171/CASE23338
PMCID: PMC10555560
PMID: 37728296
url
https://doi.org/10.3171/CASE23338View
Published (Version of record) Open Access

Abstract

BACKGROUND Malignant triton tumors (MTTs) are a rare and aggressive type of malignant peripheral nerve sheath tumor identified histologically by focal rhabdomyoblastic differentiation. OBSERVATIONS A 37-year-old female with a prior history of Hodgkin lymphoma presented with acute-onset confusion, cognitive deficits, and weakness. Brain magnetic resonance imaging revealed a hemorrhagic intracranial mass later confirmed to be a malignant triton tumor. The patient underwent two resections and several courses of chemoradiation for multiple tumor recurrences and metastases. Unfortunately, despite extensive treatment, she died 2 years after initial presentation from complications of this tumor. Her overall survival (OS) of 26.7 months was double that reported in historical cohorts (OS ∼13 mos). LESSONS The diagnosis and treatment of central nervous system MTTs are difficult and require a multidisciplinary team of neurosurgeons, radiologists, and oncologists. Histopathological analysis is required for confirmation of diagnosis. Gross-total resection of tumor and adjuvant radiation therapy have been shown to give patients the highest rates of survival and improved outcomes. Further studies and clinical trials are warranted to investigate the efficacy of chemotherapeutic agents like temozolomide, bevacizumab, and abemaciclib.

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