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

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...

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Autores principales: Zamudio-Coronado, K. Walkiria, Zohdy, Youssef M, Maldonado, Justin, Pradilla, Gustavo, Garzon-Muvdi, Tomas
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association of Neurological Surgeons 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10555614/
https://www.ncbi.nlm.nih.gov/pubmed/37728248
http://dx.doi.org/10.3171/CASE23287
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author Zamudio-Coronado, K. Walkiria
Zohdy, Youssef M
Maldonado, Justin
Pradilla, Gustavo
Garzon-Muvdi, Tomas
author_facet Zamudio-Coronado, K. Walkiria
Zohdy, Youssef M
Maldonado, Justin
Pradilla, Gustavo
Garzon-Muvdi, Tomas
author_sort Zamudio-Coronado, K. Walkiria
collection PubMed
description 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.
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spelling pubmed-105556142023-10-07 Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case Zamudio-Coronado, K. Walkiria Zohdy, Youssef M Maldonado, Justin Pradilla, Gustavo Garzon-Muvdi, Tomas J Neurosurg Case Lessons Case Lesson 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. American Association of Neurological Surgeons 2023-09-04 /pmc/articles/PMC10555614/ /pubmed/37728248 http://dx.doi.org/10.3171/CASE23287 Text en © 2023 The authors https://creativecommons.org/licenses/by-nc-nd/4.0/CC BY-NC-ND 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) )
spellingShingle Case Lesson
Zamudio-Coronado, K. Walkiria
Zohdy, Youssef M
Maldonado, Justin
Pradilla, Gustavo
Garzon-Muvdi, Tomas
Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case
title Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case
title_full Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case
title_fullStr Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case
title_full_unstemmed Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case
title_short Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case
title_sort sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. illustrative case
topic Case Lesson
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10555614/
https://www.ncbi.nlm.nih.gov/pubmed/37728248
http://dx.doi.org/10.3171/CASE23287
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