Case Report: Giant Paraganglioma of the Skull Base With Two Somatic Mutations in
Head and neck paraganglioma (HNPGL)
SDHB gene
alpha blockade
catecholamine
multidisciplinary approach
neuroendochrine tumor
paraganglioglioma
rehabilitation
Journal
Frontiers in endocrinology
ISSN: 1664-2392
Titre abrégé: Front Endocrinol (Lausanne)
Pays: Switzerland
ID NLM: 101555782
Informations de publication
Date de publication:
2022
2022
Historique:
received:
18
01
2022
accepted:
18
03
2022
entrez:
2
5
2022
pubmed:
3
5
2022
medline:
4
5
2022
Statut:
epublish
Résumé
Head and neck paragangliomas (HNPGLs) are neuroendocrine tumors. They arise from the parasympathetic ganglia and can be either sporadic or due to hereditary syndromes (up to 40%). Most HNPGLs do not produce significant amounts of catecholamines. We report a case of a giant paraganglioma of the skull base with an unusually severe presentation secondary to excessive release of norepinephrine, with a good outcome considering the severity of disease. A 39-year-old Caucasian woman with no prior medical history was found unconscious and emaciated in her home. In the intensive care unit (ICU) the patient was treated for multi-organ failure with multiple complications and difficulties in stabilizing her blood pressure with values up to 246/146 mmHg. She was hospitalized in the ICU for 72 days and on the 31
Identifiants
pubmed: 35498434
doi: 10.3389/fendo.2022.857504
pmc: PMC9044027
doi:
Substances chimiques
Normetanephrine
0J45DE6B88
SDHB protein, human
EC 1.3.5.1
Succinate Dehydrogenase
EC 1.3.99.1
PTEN Phosphohydrolase
EC 3.1.3.67
PTEN protein, human
EC 3.1.3.67
Labetalol
R5H8897N95
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
857504Informations de copyright
Copyright © 2022 Main, Benndorf, Feldt-Rasmussen, Fugleholm, Kistorp, Loya, Poulsgaard, Rasmussen, Rossing, Sølling and Klose.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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