Atypical teratoid rhabdoid tumor (AT/RT) of the central nervous system has been recently described as a distinct clinicopathological entity with characteristic morphologic, immunophenotypic and molecular characteristics. AT/RT typically involves the posterior fossa of the pediatric population. Supratentorial AT/RT is exceedingly rare. In this report, we describe a very unusual case of a child who presented with signs and symptoms suggestive of leptomeningitis. However, imaging studies and histologic findings showed plaque-like AT/RT involving the leptomeninges of the cerebrum, cerebellum, and spinal cord. The disease proved to be rapidly fatal and resulted in the patient's death within approximately two weeks. To our knowledge, this is the first case of primary leptomeningeal AT/RT involving the supratentorial leptomeninges.
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Background: Atypical teratoid rhabdoid tumor (ATRT) is the most common malignant brain tumor in infants, and more than 60% of children with ATRT die from their tumor. ATRT is associated with mutational inactivation/deletion of , a member of the SWI/SNF chromatin remodeling complex, suggesting that epigenetic events play a critical role in tumor development and progression. Moreover, disruption of SWI/SNF allows unopposed activity of epigenetic repressors, which contribute to tumorigenicity.
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November 2024
Department of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany.
Article Synopsis
- Pediatric intracranial tumors at the skull base are rare, challenging to treat due to complex anatomy, and lack substantial clinical evidence, prompting a study on surgical approaches and outcomes.
- The study involved 12 children under 18 who underwent skull base surgery from 2017 to 2023, analyzing demographics, tumor details, surgical methods, and survival rates.
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Atypical teratoid/rhabdoid tumors (AT/RTs) of the central nervous system (CNS) are rare and aggressive, typically occurring in early childhood or infancy, with adult cases being extremely rare. These tumors are associated with the inactivation of the integrase interactor 1 (INI1) gene. The prognosis is poor, worsening significantly if metastasis is detected at diagnosis.
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Department of Neurological Surgery, Weill Cornell Medicine, New York, NY 10065, USA.
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