Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case

• Authors: K. Walkiria Zamudio-Coronado, Emory University; Youssef M. Zohdy, Emory University; Justin Maldonado, Emory University; Gustavo Pradilla, Emory University; Tomas Garzon-Muvdi, Emory University
• Journal Title: Journal of Neurosurgery: Case Lessons
• Volume: Volume 6, Number 10
• Publisher: AANS | 2023-09-01
• Type of Work: Article | Final Publisher PDF
• Publisher DOI: http://doi.org/10.3171/CASE23287
• Subjects: Biology, Neuroscience; Health Sciences, Medicine and Surgery
• Keywords: ATRT; adult; sellar ATRT; sellar atypical teratoid/rhabdoid tumor

Abstract

BACKGROUND Atypical teratoid/rhabdoid tumor (ATRT) is a rare, highly aggressive central nervous system tumor predominantly found in children. Limited information exists on ATRT in adults, posing challenges in diagnosis and treatment. This study presents the case of an adult patient with ATRT in the sellar region and explores the impact of different treatment regimens on patient survival. OBSERVATIONS A 60-year-old female with an ATRT underwent resection of the tumor, followed by adjuvant chemoradiotherapy. Molecular genetic analysis revealed compound heterozygous SMARCB1 point mutations. Survival analysis was performed on previously published adult ATRT cases, comparing treatment approaches. The cohort’s overall median survival was 6 months, with patients receiving combined chemoradiotherapy showing the longest median survival of 23.5 months. Statistical analysis demonstrated a significant difference in survival between patients treated with surgery alone and those receiving surgery followed by chemoradiotherapy (p < 0.001). However, no significant difference was observed between patients treated with surgery alone and those with postoperative radiotherapy (p = 0.105). LESSONS Early initiation of adjuvant chemoradiotherapy following surgery improves survival outcomes in adult patients with ATRT. Because of limited data on standardized treatment protocols for adults with ATRT, further research and larger-scale studies are needed to establish effective treatment guidelines for this population.