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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2021; 40(02): e200-e206
DOI: 10.1055/s-0040-1719002
Case Report | Relato de Caso

Jugular Foramen's Paraganglioma in a Patient with Von Hippel-Lindau Disease: Case Report

Paraganglioma do forame jugular em paciente com doença de Von Hippel-Lindau: Relato de caso
Barbara Casalecchi Pereira 
1   Universidade Municipal de São Caetano do Sul, São Paulo, SP, Brazil
,
Julia Dallana Aznar 
1   Universidade Municipal de São Caetano do Sul, São Paulo, SP, Brazil
,
Amélia Limongi Zambon 
1   Universidade Municipal de São Caetano do Sul, São Paulo, SP, Brazil
,
Diogo Fabricio Coelho de Melo 
2   Hospital Heliópolis, São Paulo, SP, Brazil
,
Marcelo Nery Silva 
3   Neurosurgery Service, Hospital Heliópolis, São Paulo, SP, Brazil
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Abstract

Glomus jugular tumors, also known as paragangliomas (PGLs), are rare and related to several clinical syndromes described. These are located in the carotid body, the jugular glomus, the tympanic glomus and the vagal glomus. The symptoms are directly related to the site of involvement and infiltration. These lesions have slow growth, are generally benign and hypervascularized, have a peak incidence between the age of 30 to 50 years old; however, when associated with hereditary syndromes, they tend to occur a decade earlier. Several familial hereditary syndromes are associated with PGLs, including Von Hippel-Lindau disease (VHL) in < 10% of the cases. The diagnosis and staging of PGLs are based on imaging and functional exams (bone window computed tomography [CT] with a “ground moth” pattern and magnetic resonance imaging (MRI) with a “salt and pepper” pattern). The cerebral angiography is a prerequisite in patients with extremely vascularized lesions, whose preoperative embolization is necessary. The histopathological finding of cell clusters called “Zellballen” is a characteristic of PGLs. Regarding the jugular foramen, the combination of two or three surgical approaches may be necessary: (1) lateral group, approaches through the mastoid; (2) posterior group, through the retrosigmoid access and its variants; and (3) anterior group, centered on the tympanic and petrous bone. In the present paper, we report a case of PGL of the jugular foramen operated on a young female patient who underwent a surgery with a diagnosis of Von Hippel-Lindau Disease (VHL) at the Neurosurgery Service of the Hospital Heliópolis, São Paulo, state of São Paulo, Brazil in 2018, by the lateral and posterior combined route.

Resumo

Os tumores do glomus jugular, conhecido também como paragangliomas (PGLs), são raros e se correlacionam com várias síndromes clínicas descritas. Estes localizam-se no corpo carotídeo, na veia jugular, no ouvido médio e no corpo vagal. Os sintomas são diretamente relacionados ao sítio de envolvimento e infiltração. Essas lesões têm crescimento lento, geralmente são benignos e hipervascularizados, têm pico de incidência entre 30 e 50 anos de idade; contudo, quando associados a síndromes hereditárias, tendem a ocorrer uma década mais cedo. Várias síndromes hereditárias familiares estão associadas aos PGLs, dentre elas a Doença de Von Hippel-Lindau (VHL) em < 10% dos casos. O diagnóstico e estadiamento dos PGLs assentam-se na realização de exames imaginológicos e funcionais (tomografia computadorizada [TC] da janela óssea com padrão “traça moída” e ressonância magnética [RM] com padrão “sal e pimenta”) e angiografia cerebral como pré-requisito em pacientes com lesões extremamente vascularizadas, cuja embolização pré-operatória é necessária. O achado histopatológico de aglomerados celulares denominados “Zellballen” é característico dos PGLs. Em relação ao forame jugular, a combinação de dois ou três acessos cirúrgicos pode ser necessária: (1) grupo lateral, abordagens através da mastoide; (2) grupo posterior, através do acesso retrossigmóide e suas variantes; e (3) grupo anterior, centrado no osso timpânico e petroso. No presente trabalho, relatamos um caso de PGL do forame jugular em paciente jovem do sexo feminino operada com diagnóstico de Doença de Von Hippel-Lindau (VHL) no serviço de neurocirurgia do Hospital Heliópolis, São Paulo, SP, Brasil, em 2018 por via combinada lateral e posterior.

Keywords

glomus - parangliomas - von hippel-lindau

Palavras-chave

glomus - parangliomas - von hippel-lindau

Note

Institution where the Case Report was held: Hospital Heliópolis, São Paulo, state of São Paulo, Brazil




Publication History

Received: 11 May 2020

Accepted: 24 August 2020

Article published online:
26 November 2020

© 2020. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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