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Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review

BACKGROUND: Hemangioblastomas (HBs) typically present with benign behavior and are most commonly found in the posterior fossa. Multiple central nervous system (CNS) HBs are usually associated with von Hippel–Lindau disease, and leptomeningeal dissemination of sporadic HBs is extremely rare. A review...

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Autores principales: Dantas, François, Raso, Jair Leopoldo, Braga, Patrícia Salomé Gouvea, Botelho, Ricardo Vieira, Dantas, Fernando Luiz Rolemberg
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Scientific Scholar 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9479570/
https://www.ncbi.nlm.nih.gov/pubmed/36128137
http://dx.doi.org/10.25259/SNI_304_2022
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author Dantas, François
Raso, Jair Leopoldo
Braga, Patrícia Salomé Gouvea
Botelho, Ricardo Vieira
Dantas, Fernando Luiz Rolemberg
author_facet Dantas, François
Raso, Jair Leopoldo
Braga, Patrícia Salomé Gouvea
Botelho, Ricardo Vieira
Dantas, Fernando Luiz Rolemberg
author_sort Dantas, François
collection PubMed
description BACKGROUND: Hemangioblastomas (HBs) typically present with benign behavior and are most commonly found in the posterior fossa. Multiple central nervous system (CNS) HBs are usually associated with von Hippel–Lindau disease, and leptomeningeal dissemination of sporadic HBs is extremely rare. A review of the literature identified 30 cases of leptomeningeal dissemination of sporadic HBs previously published in the literature. CASE DESCRIPTION: We report the case of a patient who was diagnosed with multiple CNS HBs with aggressive progression 6 years after resection of a posterior fossa HB. He underwent multiple surgeries and died 4 years after the diagnosis of the first spinal dissemination. CONCLUSION: Dissemination of sporadic HBs is rare and aggressive disease evolution is usually observed. Further studies are necessary to determine the optimal therapeutic options.
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spelling pubmed-94795702022-09-19 Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review Dantas, François Raso, Jair Leopoldo Braga, Patrícia Salomé Gouvea Botelho, Ricardo Vieira Dantas, Fernando Luiz Rolemberg Surg Neurol Int Case Report BACKGROUND: Hemangioblastomas (HBs) typically present with benign behavior and are most commonly found in the posterior fossa. Multiple central nervous system (CNS) HBs are usually associated with von Hippel–Lindau disease, and leptomeningeal dissemination of sporadic HBs is extremely rare. A review of the literature identified 30 cases of leptomeningeal dissemination of sporadic HBs previously published in the literature. CASE DESCRIPTION: We report the case of a patient who was diagnosed with multiple CNS HBs with aggressive progression 6 years after resection of a posterior fossa HB. He underwent multiple surgeries and died 4 years after the diagnosis of the first spinal dissemination. CONCLUSION: Dissemination of sporadic HBs is rare and aggressive disease evolution is usually observed. Further studies are necessary to determine the optimal therapeutic options. Scientific Scholar 2022-08-12 /pmc/articles/PMC9479570/ /pubmed/36128137 http://dx.doi.org/10.25259/SNI_304_2022 Text en Copyright: © 2022 Surgical Neurology International https://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, transform, 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
Dantas, François
Raso, Jair Leopoldo
Braga, Patrícia Salomé Gouvea
Botelho, Ricardo Vieira
Dantas, Fernando Luiz Rolemberg
Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review
title Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review
title_full Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review
title_fullStr Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review
title_full_unstemmed Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review
title_short Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review
title_sort aggressive dissemination of central nervous system hemangioblastoma without association with von hippel–lindau disease: a case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9479570/
https://www.ncbi.nlm.nih.gov/pubmed/36128137
http://dx.doi.org/10.25259/SNI_304_2022
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