Jugular Foramen's Paraganglioma in a Patient with Von Hippel-Lindau Disease: Case Report
Arq. bras. neurocir
;
40(2): 200-205, 15/06/2021.
Article
in English
| LILACS
| ID: biblio-1362264
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 themastoid; (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 patientwho underwent a surgery with a diagnosis ofVonHippel-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.
Full text:
Available
Index:
LILACS (Americas)
Main subject:
Paraganglioma
/
Skull Base Neoplasms
/
Jugular Foramina
Type of study:
Etiology study
Limits:
Adult
/
Female
/
Humans
Language:
English
Journal:
Arq. bras. neurocir
Journal subject:
Cirurgia
/
Neurosurgery
Year:
2021
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Hospital Heliópolis/BR
/
Neurosurgery Service, Hospital Heliópolis/BR
/
Universidade Municipal de São Caetano do Sul/BR
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