神经系统的非典型畸胎/横纹肌瘤瘤（AT/RT）是一种罕见且高度恶性的肿瘤，主要影响儿童，并于1996年首次被确定为一种病理实体，并于2000年被纳入世界卫生组织对中枢神经系统肿瘤的分类。AT/RT在成人中更为罕见，并与更差的预后相关。本研究旨在分析成人肿瘤不同部位的特征。对成人非典型畸胎/横纹肌瘤的文献进行了全面和详细的回顾。分析和比较与肿瘤部位相关的人口统计学、处理和结果数据；并讨论了组织病理学和分子特征。此外，我们还增加了我们个人的案例，该案例具有大脑半球定位，并在完全切除和辅助放疗后显示了103个月的无进展生存，显示了一种奇特的组织病理学模式。AT/RT在平均部位上主要影响女性，包括颅内和脊柱内，以及所有的垂体区病例。在侧面部位，大部分实现了完全切除。联合放射治疗和化学治疗是所有肿瘤部位最常采用的辅助治疗，并与更好的预后相关。术后死亡仅在垂体区定位中报道，而脑半球病例显示了最佳总生存率。根据其位置，AT/RT显示了不同和独特的特征，这些特征显著影响结果；对它们的准确了解有助于神经外科医师制定最佳治疗策略的计划。版权所有© 2023 作者。由Elsevier Inc.出版。保留所有权利。

Atypical Teratoid/Rhabdoid Tumor (AT/RT) of the nervous system is a rare and highly malignant neoplasm, mainly affecting children, first recognized as a pathological entity in 1996 and added to the WHO Classification of the Tumors of the Central Nervous System in 2000. AT/RT is even more rare among adults and is associated to worse prognosis. The aim of the present study was to analyze the different tumor features according to the location in adults.A comprehensive and detailed literature review of Atypical Teratoid/Rhabdoid Tumours in adults was made. The demographic, management and outcome data associated to tumor location were analysed and compared; histopathological and molecular features were also discussed. Furthermore, we added our personal case with brain hemispheric localization and reporting a progression-free survival of 103 months after gross total resection and adjuvant radiotherapy and showing a peculiar histopathological pattern.Female 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 than median localizations. Combined radio- 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, while brain hemispheric cases show the best overall survival.AT/RT show different and peculiar features according to their location which significantly affect the outcome; their exact knowledge helps the neurosurgeon in the planning of the best strategy of treatment.Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.