Clinical diagnostic and radiographic features of recurrent intracranial malignant triton tumor in an adult: illustrative case.
malignant peripheral nerve sheath tumor
malignant triton tumor
rhabdomyosarcomatous
Journal
Journal of neurosurgery. Case lessons
ISSN: 2694-1902
Titre abrégé: J Neurosurg Case Lessons
Pays: United States
ID NLM: 9918227275606676
Informations de publication
Date de publication:
21 Aug 2023
21 Aug 2023
Historique:
received:
23
06
2023
accepted:
27
07
2023
medline:
20
9
2023
pubmed:
20
9
2023
entrez:
20
9
2023
Statut:
epublish
Résumé
Malignant triton tumors (MTTs) are a rare and aggressive type of malignant peripheral nerve sheath tumor identified histologically by focal rhabdomyoblastic differentiation. 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). 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.
Sections du résumé
BACKGROUND
BACKGROUND
Malignant triton tumors (MTTs) are a rare and aggressive type of malignant peripheral nerve sheath tumor identified histologically by focal rhabdomyoblastic differentiation.
OBSERVATIONS
METHODS
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
CONCLUSIONS
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.
Identifiants
pubmed: 37728296
doi: 10.3171/CASE23338
pii: CASE23338
pmc: PMC10555560
doi:
pii:
Types de publication
Journal Article
Langues
eng
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