CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery
DOI: 10.1055/s-0040-1718999
Case Report | Relato de Caso

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

Ocorrência simultânea de hemangioblastomas na região pineal e na haste hipofisária em paciente com doença de Von Hippel-Lindau – Relato de Caso
1  Centro Avançado de Neurocirurgia e Neurologia (CEANNE), Porto Alegre, Rio Grande do Sul, Brazil
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2  Hospital Cristo Redentor, Porto Alegre, Rio Grande do Sul, Brazil
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3  Universidade do Vale do Rio dos Sinos (UNISINOS), São Leopoldo, Rio Grande do Sul, Brazil
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4  Experimental Pathology, Mestrado Profissional em Odontologia Clínica, Universidade Positivo, Curitiba, Paraná, Brazil
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5  Department of Neurology, Division of Neurosurgery, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, Brazil
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5  Department of Neurology, Division of Neurosurgery, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, Brazil
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5  Department of Neurology, Division of Neurosurgery, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), São Paulo, Brazil
› Author Affiliations

Abstract

Introduction 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 neuron-specific 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.

Resumo

Introdução 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.



Publication History

Received: 12 June 2020

Accepted: 24 August 2020

Publication Date:
26 November 2020 (online)

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