Journal article
Clinical diagnostic and radiographic features of recurrent intracranial malignant triton tumor in an adult: illustrative case
Journal of Neurosurgery: Case Lessons, Vol.6(8), CASE23338
08/21/2023
DOI: 10.3171/CASE23338
PMCID: PMC10555560
PMID: 37728296
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.
Details
- Title: Subtitle
- Clinical diagnostic and radiographic features of recurrent intracranial malignant triton tumor in an adult: illustrative case
- Creators
- Ajmain Chowdhury - University of IowaAlex Belzer - Carver College of Medicine, University of Iowa, Iowa City, IowaNahom Teferi - Departments of NeurosurgeryHend Al-Kaylani - University of IowaJuan Vivanco-Suarez - Departments of NeurosurgeryAssim Saad Eddin - RadiologyKathryn Eschbacher - Pathology, andMohammed Milhem - University of IowaPatrick Hitchon - Departments of Neurosurgery
- Resource Type
- Journal article
- Publication Details
- Journal of Neurosurgery: Case Lessons, Vol.6(8), CASE23338
- DOI
- 10.3171/CASE23338
- PMID
- 37728296
- PMCID
- PMC10555560
- NLM abbreviation
- J Neurosurg Case Lessons
- ISSN
- 2694-1902
- eISSN
- 2694-1902
- Language
- English
- Date published
- 08/21/2023
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Neurology; Radiology; Hematology, Oncology, and Blood & Marrow Transplantation; Pathology; Orthopedics and Rehabilitation; Neurosurgery; Internal Medicine
- Record Identifier
- 9984459416202771
Metrics
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