Atypical Teratoid Rhabdoid Tumor of the Nervous System in Adults: Location-Related Features and Outcome

Background: 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. Methods: 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. Results: 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. Conclusions: 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.