• Sergio Corvino, Marialaura Del Basso De Caro, Raduan Ahmed Franca, Giuseppe Corazzelli, Rosa Della Monica, Lorenzo Chiariotti, and Francesco Maiuri.
• Department of Neuroscience and Reproductive and Odontostomatological Sciences, Neurosurgical Clinic, School of Medicine, University of Naples "Federico II", Naples, Italy.
• World Neurosurg. 2023 Nov 1; 179: e404e415e404-e415.

BackgroundAtypical teratoid/rhabdoid tumor (AT/RT) of the nervous system is a rare and highly malignant neoplasm, mainly affecting children, first recognized as a pathologic entity in 1996 and added to the World Health Organization Classification of the Tumors of the Central Nervous System in 2000. AT/RT is even rarer among adults and is associated with a worse prognosis. The aim of the present study was to analyze the different tumor features according to the location in adults.MethodsA comprehensive and detailed literature review of AT/RTs in adults was made. The demographic, management, and outcome data associated with tumor location were analyzed and compared; histopathologic and molecular features were also discussed. Furthermore, we added our personal case with brain hemispheric localization and reported a progression-free survival of 103 months after gross total resection and adjuvant radiotherapy showing a peculiar histopathologic pattern.ResultsFemale sex is mainly affected by AT/RT on median localizations, both intracranial and spinal, and by all sellar region cases. Gross total resection is mainly achieved among lateral compared with median localizations. Combined radiotherapy and chemotherapy is the most adopted adjuvant treatment in all tumor localizations and is related to better outcome. Postoperative death is reported only among sellar region localizations, whereas brain hemispheric cases show the best overall survival.ConclusionsAT/RTs show different and peculiar features according to their location, which significantly affects the outcome; precise knowledge of them helps the neurosurgeon in planning the best strategy for treatment.Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.

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