• Garret P Greeneway, Paul S Page, Viharkumar Patel, and Azam S Ahmed.
• Department of Neurological Surgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA. Electronic address: gpgreeneway@gmail.com.
• World Neurosurg. 2021 Jan 1; 145: 57-63.

BackgroundAtypical teratoid/rhabdoid tumor (AT/RT) is a rare tumor that is most frequently encountered in the pediatric patient population. AT/RT accounts for approximately 1%-2% of all pediatric central nervous system tumors and roughly 10%-20% of tumors in patients younger than 3 years of age. While AT/RT has been encountered in the adult population, the vast majority of the cases reported occur in the supratentorial space. In the existing literature, only 3 adult cases that arise from the cerebellum have ever been reported.Case DescriptionA 38-year-old female presented with 6 months of worsening nausea, emesis, vertigo, diplopia, and coordination difficulty. Magnetic resonance imaging revealed a T1 avidly contrast-enhancing mass, composed of both cystic and solid areas, extending from the cerebellum into the fourth ventricle. Following a gross total resection, surgical pathology was consistent with AT/RT, with tumor cell loss of integrase interactor-1 (INI-1) observed via immunohistochemical staining.ConclusionsThis case represents just the fourth ever reported case of AT/RT arising from the cerebellum in an adult and the oldest reported age to date of a cerebellar AT/RT occurring in a female. Due to the paucity of reported adult AT/RT cases, little is known about adults with AT/RT. Further reports will function to improve the general understanding of AT/RT in the adult population.Copyright © 2020 Elsevier Inc. All rights reserved.

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