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Primary Central Nervous System Fibrosarcoma

We report a rare case of a young female with primary brain fibrosarcoma, and to the best of our knowledge, we believe that only <50 cases have been reported or described worldwide so far. Fibrosarcoma is a malignant neoplasm, in which histologically the predominant cells are fibroblasts that divi...

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Autores principales: Vinodh, VP, Harun, Rahmat, Sellamuthu, Pulivendhan, Kandasamy, Regunath
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5602233/
https://www.ncbi.nlm.nih.gov/pubmed/28936084
http://dx.doi.org/10.4103/jnrp.jnrp_165_17
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author Vinodh, VP
Harun, Rahmat
Sellamuthu, Pulivendhan
Kandasamy, Regunath
author_facet Vinodh, VP
Harun, Rahmat
Sellamuthu, Pulivendhan
Kandasamy, Regunath
author_sort Vinodh, VP
collection PubMed
description We report a rare case of a young female with primary brain fibrosarcoma, and to the best of our knowledge, we believe that only <50 cases have been reported or described worldwide so far. Fibrosarcoma is a malignant neoplasm, in which histologically the predominant cells are fibroblasts that divide excessively without cellular control and they can invade local tissues or metastasize. Primary central nervous system fibrosarcomas are very aggressive neoplasms and generally have a poor prognosis. This tumor is either from sarcomatous transformation of a meningioma or arises de novo within the brain parenchyma. Our patient, a 48-year-old woman, who presented with progressive speech disorder over the period of 4 months, showed a left temporoparietal lesion with surrounding edema and local mass effect. Total surgical resection was achieved. Histopathology revealed classical fibrosarcoma features and secondary screening revealed no other distant lesion as diagnosis of primary brain fibrosarcoma was established. This case is deemed to be extremely rare because most reports claim that recurrence is within 6 months with poor prognosis; however, this patient is currently recurrence-free at 3 years. This would suggest of the possibility for a relook into this disease's course and recurrence rate when complete excision is achieved. Due to extreme rarity of these tumors, more comparative studies will be needed to improve the disease outcome.
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spelling pubmed-56022332017-09-21 Primary Central Nervous System Fibrosarcoma Vinodh, VP Harun, Rahmat Sellamuthu, Pulivendhan Kandasamy, Regunath J Neurosci Rural Pract Case Report We report a rare case of a young female with primary brain fibrosarcoma, and to the best of our knowledge, we believe that only <50 cases have been reported or described worldwide so far. Fibrosarcoma is a malignant neoplasm, in which histologically the predominant cells are fibroblasts that divide excessively without cellular control and they can invade local tissues or metastasize. Primary central nervous system fibrosarcomas are very aggressive neoplasms and generally have a poor prognosis. This tumor is either from sarcomatous transformation of a meningioma or arises de novo within the brain parenchyma. Our patient, a 48-year-old woman, who presented with progressive speech disorder over the period of 4 months, showed a left temporoparietal lesion with surrounding edema and local mass effect. Total surgical resection was achieved. Histopathology revealed classical fibrosarcoma features and secondary screening revealed no other distant lesion as diagnosis of primary brain fibrosarcoma was established. This case is deemed to be extremely rare because most reports claim that recurrence is within 6 months with poor prognosis; however, this patient is currently recurrence-free at 3 years. This would suggest of the possibility for a relook into this disease's course and recurrence rate when complete excision is achieved. Due to extreme rarity of these tumors, more comparative studies will be needed to improve the disease outcome. Medknow Publications & Media Pvt Ltd 2017-08 /pmc/articles/PMC5602233/ /pubmed/28936084 http://dx.doi.org/10.4103/jnrp.jnrp_165_17 Text en Copyright: © 2017 Journal of Neurosciences in Rural Practice 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
Vinodh, VP
Harun, Rahmat
Sellamuthu, Pulivendhan
Kandasamy, Regunath
Primary Central Nervous System Fibrosarcoma
title Primary Central Nervous System Fibrosarcoma
title_full Primary Central Nervous System Fibrosarcoma
title_fullStr Primary Central Nervous System Fibrosarcoma
title_full_unstemmed Primary Central Nervous System Fibrosarcoma
title_short Primary Central Nervous System Fibrosarcoma
title_sort primary central nervous system fibrosarcoma
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5602233/
https://www.ncbi.nlm.nih.gov/pubmed/28936084
http://dx.doi.org/10.4103/jnrp.jnrp_165_17
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