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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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Detalles Bibliográficos
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
Descripción
Sumario: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.