Pheochromocytomas and paragangliomas in von Hippel-Lindau disease: not a needle in a haystack.
frequency
paraganglioma
pheochromocytoma
systematic
von Hippel–Lindau
Journal
Endocrine connections
ISSN: 2049-3614
Titre abrégé: Endocr Connect
Pays: England
ID NLM: 101598413
Informations de publication
Date de publication:
27 Oct 2021
27 Oct 2021
Historique:
received:
14
09
2021
accepted:
30
09
2021
pubmed:
2
10
2021
medline:
2
10
2021
entrez:
1
10
2021
Statut:
epublish
Résumé
Pheochromocytomas are a hallmark feature of von Hippel-Lindau disease (vHL). To our knowledge, this is the first systematic review with meta-analysis evaluating the frequency of pheochromocytomas and/or paragangliomas (PPGLs) in patients with vHL, as well as among patients with different vHL subtypes. Systematic review with meta-analysis. We searched on MEDLINE, Scopus, and Web of Science. We included primary studies assessing participants with vHL and reporting on the frequency of PPGL. We performed random-effects meta-analysis to quantitatively assess the frequency of PPGL, followed by meta-regression and subgroup analysis. Risk of bias analysis was performed to assess primary studies' methodological quality. We included 80 primary studies. In 4263 patients with vHL, the pooled frequency of PPGL was 19.4% (95% CI = 15.9-23.6%, I2 = 86.1%). The frequency increased to 60.0% in patients with vHL type 2 (95% CI = 53.4-66.3%, I2 = 54.6%) and was determined to be of 58.2% in patients with vHL type 2A (95% CI = 49.7-66.3%, I2 = 36.2%), compared to 49.8% in vHL type 2B (95% CI = 39.9-59.7%, I2 = 42.7%), and 84.1% in vHL type 2C (95% CI = 75.1-93.1%, I2 = 0%). In meta-regression analysis, more recent studies were associated with a higher frequency of PPGL. All studies had at least one internal validity item classified as 'high risk of bias,' with 13% studies having low risk of bias in all external validity items. PPGLs are a common manifestation of vHL. Despite methodological limitations and differences across primary studies, our results point to the importance of PPGL screening in patients with vHL.
Identifiants
pubmed: 34596579
doi: 10.1530/EC-21-0294
pii: EC-21-0294
pmc: PMC8630766
doi:
pii:
Types de publication
Journal Article
Review
Langues
eng
Pagination
R293-R304Références
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