Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack
Autor(a) principal: | |
---|---|
Data de Publicação: | 2021 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://hdl.handle.net/10216/155596 |
Resumo: | Objective: 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. Design: Systematic review with meta-analysis. Methods: 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. Results: 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. Conclusions: 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. |
id |
RCAP_70d0561943b76c484b9c4992c89e20ec |
---|---|
oai_identifier_str |
oai:repositorio-aberto.up.pt:10216/155596 |
network_acronym_str |
RCAP |
network_name_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository_id_str |
7160 |
spelling |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystackFrequencyParagangliomaPheochromocytomaSystematicVon Hippel-LindauObjective: 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. Design: Systematic review with meta-analysis. Methods: 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. Results: 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. Conclusions: 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.BioScientifica20212021-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://hdl.handle.net/10216/155596eng2049-361410.1530/EC-21-0294Castro-Teles, JSousa-Pinto, BRebelo, SPignatelli, Dinfo:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-12-16T06:00:57Zoai:repositorio-aberto.up.pt:10216/155596Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T00:54:25.478351Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack |
title |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack |
spellingShingle |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack Castro-Teles, J Frequency Paraganglioma Pheochromocytoma Systematic Von Hippel-Lindau |
title_short |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack |
title_full |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack |
title_fullStr |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack |
title_full_unstemmed |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack |
title_sort |
Pheochromocytomas and paragangliomas in von hippel–lindau disease: Not a needle in a haystack |
author |
Castro-Teles, J |
author_facet |
Castro-Teles, J Sousa-Pinto, B Rebelo, S Pignatelli, D |
author_role |
author |
author2 |
Sousa-Pinto, B Rebelo, S Pignatelli, D |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Castro-Teles, J Sousa-Pinto, B Rebelo, S Pignatelli, D |
dc.subject.por.fl_str_mv |
Frequency Paraganglioma Pheochromocytoma Systematic Von Hippel-Lindau |
topic |
Frequency Paraganglioma Pheochromocytoma Systematic Von Hippel-Lindau |
description |
Objective: 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. Design: Systematic review with meta-analysis. Methods: 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. Results: 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. Conclusions: 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. |
publishDate |
2021 |
dc.date.none.fl_str_mv |
2021 2021-01-01T00:00:00Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://hdl.handle.net/10216/155596 |
url |
https://hdl.handle.net/10216/155596 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
2049-3614 10.1530/EC-21-0294 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
BioScientifica |
publisher.none.fl_str_mv |
BioScientifica |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
repository.mail.fl_str_mv |
|
_version_ |
1799136431889711104 |