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Primary Intracranial Synovial Sarcoma

Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial s...

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Autores principales: Patel, Mohit, Li, Luyuan, Nguyen, Ha Son, Doan, Ninh, Sinson, Grant, Mueller, Wade
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4876212/
https://www.ncbi.nlm.nih.gov/pubmed/27247811
http://dx.doi.org/10.1155/2016/5608315
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author Patel, Mohit
Li, Luyuan
Nguyen, Ha Son
Doan, Ninh
Sinson, Grant
Mueller, Wade
author_facet Patel, Mohit
Li, Luyuan
Nguyen, Ha Son
Doan, Ninh
Sinson, Grant
Mueller, Wade
author_sort Patel, Mohit
collection PubMed
description Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression.
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spelling pubmed-48762122016-05-31 Primary Intracranial Synovial Sarcoma Patel, Mohit Li, Luyuan Nguyen, Ha Son Doan, Ninh Sinson, Grant Mueller, Wade Case Rep Neurol Med Case Report Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. Hindawi Publishing Corporation 2016 2016-05-09 /pmc/articles/PMC4876212/ /pubmed/27247811 http://dx.doi.org/10.1155/2016/5608315 Text en Copyright © 2016 Mohit Patel et al. https://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
Patel, Mohit
Li, Luyuan
Nguyen, Ha Son
Doan, Ninh
Sinson, Grant
Mueller, Wade
Primary Intracranial Synovial Sarcoma
title Primary Intracranial Synovial Sarcoma
title_full Primary Intracranial Synovial Sarcoma
title_fullStr Primary Intracranial Synovial Sarcoma
title_full_unstemmed Primary Intracranial Synovial Sarcoma
title_short Primary Intracranial Synovial Sarcoma
title_sort primary intracranial synovial sarcoma
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4876212/
https://www.ncbi.nlm.nih.gov/pubmed/27247811
http://dx.doi.org/10.1155/2016/5608315
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