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Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review

BACKGROUND: Hemangiopericytoma and solitary fibrous tumor (HPC/SFT) are considered to be one category according to the WHO 2016 classification of central nervous system tumors. HPC/SFT are subdivided into infantile (congenital) and adult type. Both are extremely rare entities, with little knowledge...

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Autores principales: Giakoumettis, Dimitrios, Nikas, Ioannis, Stefanaki, Kalliopi, Kattamis, Antonis, Sfakianos, George, Themistocleous, Marios S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Scientific Scholar 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6744783/
https://www.ncbi.nlm.nih.gov/pubmed/31528413
http://dx.doi.org/10.25259/SNI-85-2019
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author Giakoumettis, Dimitrios
Nikas, Ioannis
Stefanaki, Kalliopi
Kattamis, Antonis
Sfakianos, George
Themistocleous, Marios S.
author_facet Giakoumettis, Dimitrios
Nikas, Ioannis
Stefanaki, Kalliopi
Kattamis, Antonis
Sfakianos, George
Themistocleous, Marios S.
author_sort Giakoumettis, Dimitrios
collection PubMed
description BACKGROUND: Hemangiopericytoma and solitary fibrous tumor (HPC/SFT) are considered to be one category according to the WHO 2016 classification of central nervous system tumors. HPC/SFT are subdivided into infantile (congenital) and adult type. Both are extremely rare entities, with little knowledge about etiology, prognosis, and optimal therapeutic strategy. CASE DESCRIPTION: A 10-day-old girl was referred to our neurosurgical department due to hypotonia, palsy of the right oculomotor nerve, and prominent frontal fontanel. Imaging studies revealed a large occupying mass in the right middle cerebral fossa and the suprasellar cisterns. Only a subtotal resection of the tumor was possible, and postoperatively, she underwent chemotherapy (CHx). After a 3-year follow-up, the girl has minimum neurologic signs and receives no medications, and she can walk when she is supported. CONCLUSION: Congenital HPC/SFT is considered to have a benign behavior with a good prognosis. Treatment with gross total resection, when it is feasible, is the key to a good prognosis and low rates of recurrence. However, there is no consensus on the therapeutic strategy of a HPC/SFT, which is difficult to be completely resected. Literature lacks a therapeutic algorithm for these tumors, and thus, more clinical studies are needed to reach a consensus.
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spelling pubmed-67447832019-09-16 Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review Giakoumettis, Dimitrios Nikas, Ioannis Stefanaki, Kalliopi Kattamis, Antonis Sfakianos, George Themistocleous, Marios S. Surg Neurol Int Case Report BACKGROUND: Hemangiopericytoma and solitary fibrous tumor (HPC/SFT) are considered to be one category according to the WHO 2016 classification of central nervous system tumors. HPC/SFT are subdivided into infantile (congenital) and adult type. Both are extremely rare entities, with little knowledge about etiology, prognosis, and optimal therapeutic strategy. CASE DESCRIPTION: A 10-day-old girl was referred to our neurosurgical department due to hypotonia, palsy of the right oculomotor nerve, and prominent frontal fontanel. Imaging studies revealed a large occupying mass in the right middle cerebral fossa and the suprasellar cisterns. Only a subtotal resection of the tumor was possible, and postoperatively, she underwent chemotherapy (CHx). After a 3-year follow-up, the girl has minimum neurologic signs and receives no medications, and she can walk when she is supported. CONCLUSION: Congenital HPC/SFT is considered to have a benign behavior with a good prognosis. Treatment with gross total resection, when it is feasible, is the key to a good prognosis and low rates of recurrence. However, there is no consensus on the therapeutic strategy of a HPC/SFT, which is difficult to be completely resected. Literature lacks a therapeutic algorithm for these tumors, and thus, more clinical studies are needed to reach a consensus. Scientific Scholar 2019-04-24 /pmc/articles/PMC6744783/ /pubmed/31528413 http://dx.doi.org/10.25259/SNI-85-2019 Text en Copyright: © 2019 Surgical Neurology International http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 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
Giakoumettis, Dimitrios
Nikas, Ioannis
Stefanaki, Kalliopi
Kattamis, Antonis
Sfakianos, George
Themistocleous, Marios S.
Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review
title Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review
title_full Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review
title_fullStr Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review
title_full_unstemmed Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review
title_short Giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: A case report and literature review
title_sort giant intracranial congenital hemangiopericytoma/solitary fibrous tumor: a case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6744783/
https://www.ncbi.nlm.nih.gov/pubmed/31528413
http://dx.doi.org/10.25259/SNI-85-2019
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