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A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child
Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system neoplasm affecting children, and isolated primary spinal involvement is extremely rare. Authors describe a case of spinal AT/RT in a 5-year-old male child presenting with rapidly progressing quadriparesis diagnosed and managed...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Medknow Publications & Media Pvt Ltd
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4991152/ https://www.ncbi.nlm.nih.gov/pubmed/27606020 http://dx.doi.org/10.4103/1817-1745.187634 |
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author | Mankotia, Dipanker Singh Tandon, Vivek Sharma, Bhawani Shankar Rajeshwari, Madhu Sharma, Mehar Chand |
author_facet | Mankotia, Dipanker Singh Tandon, Vivek Sharma, Bhawani Shankar Rajeshwari, Madhu Sharma, Mehar Chand |
author_sort | Mankotia, Dipanker Singh |
collection | PubMed |
description | Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system neoplasm affecting children, and isolated primary spinal involvement is extremely rare. Authors describe a case of spinal AT/RT in a 5-year-old male child presenting with rapidly progressing quadriparesis diagnosed and managed surgically and medically. Biopsy revealed large, rhabdoid cells with prominent nucleoli in nest and immunohistochemistry further showed loss of integrase integrator 1 expression considered to be gold standard for diagnosis. AT/RT has extremely poor prognosis with median survival being 6 months. |
format | Online Article Text |
id | pubmed-4991152 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Medknow Publications & Media Pvt Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-49911522016-09-07 A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child Mankotia, Dipanker Singh Tandon, Vivek Sharma, Bhawani Shankar Rajeshwari, Madhu Sharma, Mehar Chand J Pediatr Neurosci Case Report Atypical teratoid/rhabdoid tumor (AT/RT) is a rare central nervous system neoplasm affecting children, and isolated primary spinal involvement is extremely rare. Authors describe a case of spinal AT/RT in a 5-year-old male child presenting with rapidly progressing quadriparesis diagnosed and managed surgically and medically. Biopsy revealed large, rhabdoid cells with prominent nucleoli in nest and immunohistochemistry further showed loss of integrase integrator 1 expression considered to be gold standard for diagnosis. AT/RT has extremely poor prognosis with median survival being 6 months. Medknow Publications & Media Pvt Ltd 2016 /pmc/articles/PMC4991152/ /pubmed/27606020 http://dx.doi.org/10.4103/1817-1745.187634 Text en Copyright: © Journal of Pediatric Neurosciences http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Mankotia, Dipanker Singh Tandon, Vivek Sharma, Bhawani Shankar Rajeshwari, Madhu Sharma, Mehar Chand A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child |
title | A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child |
title_full | A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child |
title_fullStr | A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child |
title_full_unstemmed | A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child |
title_short | A case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child |
title_sort | case of primary spinal atypical teratoid/rhabdoid tumor in a 5-year-old child |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4991152/ https://www.ncbi.nlm.nih.gov/pubmed/27606020 http://dx.doi.org/10.4103/1817-1745.187634 |
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