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Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy
Atypical teratoid rhabdoid tumors (ATRTs) are rare and aggressive central nervous system tumors that infrequently arise in spinal locations in young children. Provided clinical and diagnostic suspicion is high, the histopathological diagnosis is relatively straightforward to secure by testing for th...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6815537/ https://www.ncbi.nlm.nih.gov/pubmed/31737396 http://dx.doi.org/10.1155/2019/3842835 |
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author | Refai, Fahd Al-Maghrabi, Haneen Al Trabolsi, Hassan Al-Maghrabi, Jaudah |
author_facet | Refai, Fahd Al-Maghrabi, Haneen Al Trabolsi, Hassan Al-Maghrabi, Jaudah |
author_sort | Refai, Fahd |
collection | PubMed |
description | Atypical teratoid rhabdoid tumors (ATRTs) are rare and aggressive central nervous system tumors that infrequently arise in spinal locations in young children. Provided clinical and diagnostic suspicion is high, the histopathological diagnosis is relatively straightforward to secure by testing for the characteristic loss of the tumor suppressor protein SMARCB1/INI1. Here, we describe a case of thoracic spinal ATRT in a three-year-old boy that showed characteristic aggressive progression until managed with intensive multimodal therapy to achieve durable long-term remission. In doing so, we review the histopathological features, management, and current advances in molecular biology that hold promise for personalized ATRT therapy. |
format | Online Article Text |
id | pubmed-6815537 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Hindawi |
record_format | MEDLINE/PubMed |
spelling | pubmed-68155372019-11-17 Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy Refai, Fahd Al-Maghrabi, Haneen Al Trabolsi, Hassan Al-Maghrabi, Jaudah Case Rep Pediatr Case Report Atypical teratoid rhabdoid tumors (ATRTs) are rare and aggressive central nervous system tumors that infrequently arise in spinal locations in young children. Provided clinical and diagnostic suspicion is high, the histopathological diagnosis is relatively straightforward to secure by testing for the characteristic loss of the tumor suppressor protein SMARCB1/INI1. Here, we describe a case of thoracic spinal ATRT in a three-year-old boy that showed characteristic aggressive progression until managed with intensive multimodal therapy to achieve durable long-term remission. In doing so, we review the histopathological features, management, and current advances in molecular biology that hold promise for personalized ATRT therapy. Hindawi 2019-10-13 /pmc/articles/PMC6815537/ /pubmed/31737396 http://dx.doi.org/10.1155/2019/3842835 Text en Copyright © 2019 Fahd Refai et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Refai, Fahd Al-Maghrabi, Haneen Al Trabolsi, Hassan Al-Maghrabi, Jaudah Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy |
title | Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy |
title_full | Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy |
title_fullStr | Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy |
title_full_unstemmed | Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy |
title_short | Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy |
title_sort | long-term remission of a spinal atypical teratoid rhabdoid tumor in response to intensive multimodal therapy |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6815537/ https://www.ncbi.nlm.nih.gov/pubmed/31737396 http://dx.doi.org/10.1155/2019/3842835 |
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