Co-Occurrence of Pineal-Region and Pituitary-Stalk Hemangioblastomas in a Patient Presenting with Von Hippel-Lindau Disease A Case Report
Arq. bras. neurocir
; 40(2): 190-194, 15/06/2021.
Artigo
em Inglês
| LILACS
| ID: biblio-1362260
Biblioteca responsável:
BR1.1
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 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.
Texto completo:
Disponível
Coleções:
Bases de dados internacionais
Contexto em Saúde:
ODS3 - Meta 3.8 Atingir a cobertura universal de saúde
Problema de saúde:
Arranjos de Entrega
Base de dados:
LILACS
Assunto principal:
Glândula Pineal
/
Pinealoma
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Hipófise
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Hemangioblastoma
Limite:
Adulto
/
Feminino
/
Humanos
Idioma:
Inglês
Revista:
Arq. bras. neurocir
Assunto da revista:
Cirurgia
/
Neurocirurgia
Ano de publicação:
2021
Tipo de documento:
Artigo
País de afiliação:
Brasil
Instituição/País de afiliação:
Centro Avançado de Neurocirurgia e Neurologia (CEANNE)/BR
/
Department of Neurology, Division of Neurosurgery, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo (HCFMUSP)/BR
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Experimental Pathology, Mestrado Profissional em Odontologia Clínica, Universidade Positivo/BR
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Hospital Cristo Redentor/BR
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Universidade do Vale do Rio dos Sinos (UNISINOS)/BR