Co-Occurrence of Pineal-Region and Pituitary-Stalk Hemangioblastomas in a Patient Presenting with Von Hippel-Lindau Disease - A Case Report

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorISOLAN, Gustavo Rassier
dc.contributor.authorPERONDI, Gerson Evandro
dc.contributor.authorDALLA-CORTE, Amauri
dc.contributor.authorGIOVANINI, Allan Fernando
dc.contributor.authorTELLES, Joao Paulo Mota
dc.contributor.authorRABELO, Nicollas Nunes
dc.contributor.authorFIGUEIREDO, Eberval Gadelha
dc.date.accessioned2021-12-16T14:26:12Z
dc.date.available2021-12-16T14:26:12Z
dc.date.issued2021
dc.description.abstractIntroduction Hemangioblastomas of the pineal region or pituitary stalk are extremely rare. Only two cases of hemangioblastomas involving the pineal region have been reported, and four involving the pituitary stalk. The purpose of the present manuscript is to describe an unusual case of supposed hemangioblastoma found concomitantly in the pineal region and pituitary stalk of a patient diagnosed with Von Hippel- Lindau (VHL) disease. Case Report A 35-year-old female patient with a previous diagnosis of VHL complaining of occipital headaches and balance disturbances for three weeks, who previously had a cerebellar hemangioblastoma resected. The visual characteristics of the tumor suggested a friable vascular lesion with a reddish-brown surface, and an incisional biopsy was performed. The tumor consisted of a dense vascular network surrounded by fibrous stroma abundant in reticulin and composed by both fusiform and dispersed xanthomatous cells; the immunohistochemistry was immunopositive for neuronspecific enolase and immunonegative for epithelial membranous antigen. The patient has been monitored closely for 2 years, and the supratentorial masses have not presented any volume alteration. Conclusion This rare association must be taken into account in patients with VHL disease, or at least be suspected in patients who present a thickening of the pituitary stalk and a pineal-region mass. We believe a biopsy of our asymptomatic patient could have been dangerous due to inherent complications like intraoperative bleeding. We recommend close observation of asymptomatic lesions with MRIs every six months or until the lesions become symptomatic. If the pineal-region tumor does become symptomatic, gross resection via a transcallosal approach would be ideal.eng
dc.description.abstractIntrodução Hemangioblastomas da região pineal ou da haste hipofisária são raros. Apenas 2 casos foram reportados para a pineal e 4, para a haste hipofisária. O objetivo deste artigo é relatar um caso incomum de supostos hemangioblastomas encontrados concomitantemente na pineal e na haste hipofisária em paciente com Doença de Von Hippel-Lindau (VHL). Relato de Caso Mulher de 35 anos, com diagnóstico de VHL e histórico de ressecção de hemangioblastoma cerebelar, apresentou cefaleia occipital e queixas relacionadas ao equilíbrio por 3 semanas. As características visuais do tumor sugeriam lesão vascular friável com superfície vermelho-amarronzada, sendo realizada biópsia incisional. O tumor consistia de rede vascular densa rodeada de estroma fibrótico abundante em reticulina e composto de células xantomatosas. Imunohistoquímica positive para enolase específica de neurônios (NSE) e negative para antígeno membranoso epitelial (EMA). Paciente monitorada por 2 anos, sem alteração nas massas. Conclusão Essa associação rara deve ser suspeitada em pacientes com VHL, na presença de espessamento da haste hipofisária e massa pineal. Acreditamos que uma biópsia, para a paciente assintomática, seria perigosa devido a complicações inerentes como hemorragia intra-operatória. Recomendamos observação de lesões assintomáticas com RM a cada 6 meses ou até que as lesões se tornem sintomáticas. Caso o tumor da região pineal se torne sintomático, ressecção macroscópica por via transcalosa seria ideal.
dc.description.indexWoSeng
dc.identifier.citationBRAZILIAN NEUROSURGERY-ARQUIVOS BRASILEIROS DE NEUROCIRURGIA, v.40, n.2, p.E190-E194, 2021
dc.identifier.doi10.1055/s-0040-1718999
dc.identifier.eissn2359-5922
dc.identifier.issn0103-5355
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/43889
dc.language.isoeng
dc.publisherGEORG THIEME VERLAG KGeng
dc.relation.ispartofBrazilian Neurosurgery-Arquivos Brasileiros de Neurocirurgia
dc.rightsopenAccesseng
dc.rights.holderCopyright GEORG THIEME VERLAG KGeng
dc.subjecttumoreng
dc.subjectvon hippel-lindaueng
dc.subjecthemangioblastomaeng
dc.subject.othercentral-nervous-systemeng
dc.subject.othersurgical-managementeng
dc.subject.wosSurgeryeng
dc.titleCo-Occurrence of Pineal-Region and Pituitary-Stalk Hemangioblastomas in a Patient Presenting with Von Hippel-Lindau Disease - A Case Reporteng
dc.title.alternativeOcorrência simultânea de hemangioblastomas na região pineal e na haste hipofisária em paciente com doença de Von Hippel-Lindau – Relato de Caso
dc.typearticleeng
dc.type.categoryoriginal articleeng
dc.type.versionpublishedVersioneng
dspace.entity.typePublication
hcfmusp.author.externalISOLAN, Gustavo Rassier:Ctr Avd Neurocirurgia & Neurol CEANNE, Porto Alegre, RS, Brazil
hcfmusp.author.externalPERONDI, Gerson Evandro:Hosp Cristo Redentor, Porto Alegre, RS, Brazil
hcfmusp.author.externalDALLA-CORTE, Amauri:Univ Vale Rio Sinos UNISINOS, Sao Leopoldo, RS, Brazil
hcfmusp.author.externalGIOVANINI, Allan Fernando:Univ Positivo, Expt Pathol Mestrado Profiss Odontol Clin, Curitiba, Parana, Brazil
hcfmusp.contributor.author-fmusphcJOAO PAULO MOTA TELLES
hcfmusp.contributor.author-fmusphcNICOLLAS NUNES RABELO
hcfmusp.contributor.author-fmusphcEBERVAL GADELHA FIGUEIREDO
hcfmusp.description.beginpageE190
hcfmusp.description.endpageE194
hcfmusp.description.issue2
hcfmusp.description.volume40
hcfmusp.origemWOS
hcfmusp.origem.wosWOS:000592404300005
hcfmusp.publisher.citySTUTTGARTeng
hcfmusp.publisher.countryGERMANYeng
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