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...
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| Format: | Article |
| Language: | English |
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Wiley
2019-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2019/3842835 |
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| _version_ | 1832552263840694272 |
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| author | Fahd Refai Haneen Al-Maghrabi Hassan Al Trabolsi Jaudah Al-Maghrabi |
| author_facet | Fahd Refai Haneen Al-Maghrabi Hassan Al Trabolsi Jaudah Al-Maghrabi |
| author_sort | Fahd Refai |
| collection | DOAJ |
| 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 | Article |
| id | doaj-art-e4688ca55f834963adf8004b455f112b |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2019-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-e4688ca55f834963adf8004b455f112b2025-02-03T05:59:18ZengWileyCase Reports in Pediatrics2090-68032090-68112019-01-01201910.1155/2019/38428353842835Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal TherapyFahd Refai0Haneen Al-Maghrabi1Hassan Al Trabolsi2Jaudah Al-Maghrabi3Department of Pathology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi ArabiaDepartment of Pathology, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi ArabiaOncology Department, King Faisal Specialist Hospital and Research Centre, Jeddah, Saudi ArabiaDepartment of Pathology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi ArabiaAtypical 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.http://dx.doi.org/10.1155/2019/3842835 |
| spellingShingle | Fahd Refai Haneen Al-Maghrabi Hassan Al Trabolsi Jaudah Al-Maghrabi Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy Case Reports in Pediatrics |
| 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 |
| url | http://dx.doi.org/10.1155/2019/3842835 |
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