{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T20:49:41Z","timestamp":1791233381520,"version":"4.1.0"},"reference-count":94,"publisher":"The Endocrine Society","issue":"2","license":[{"start":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T00:00:00Z","timestamp":1762128000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,1,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Paragangliomas and phaeochromocytomas (PPGLs) are rare neuroendocrine tumours with 10% to 20% of patients developing metastatic disease. The tumors exhibit a high degree of heritability, and pathogenic genetic variants have been associated with metastases.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>We aimed to investigate the association between the genotype of PPGL patients and their risk of metastatic disease, adjusting for time.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A systematic search was conducted in PubMed and Embase. The primary outcome was the rate of metastatic disease per 100 followed patient-years analyzed through meta-analyses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A significantly increased rate of metastatic disease per 100 followed patient-years was observed in all pathogenic germline variants included in the analyses, compared to the group with no identified variant. The group with no variant had a rate of 1.55 per 100 patient-years. SDHA, SDHC, SDHD, VHL, RET, NF1, and MAX had rates of 13.73, 6.27, 2.03, 2.34, 1.91, 4.11, and 9.66, respectively. The rate of SDHB is not presented as statistical heterogeneity exceeded 75%. The pathogenic somatic variant EPAS1 showed a rate of 3.82. Cluster-divided meta-analyses resulted in rates of 4.41 and 3.0 for cluster 1 and cluster 2, respectively. Meta-regression analysis revealed a 2.3-fold higher rate for the SDHB variant compared to the other cluster 1 variants.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>We present associations between genotype and metastatic disease in PPGL patients. Our results indicate that patients harboring a pathogenic genetic variant have a higher rate of metastases compared to patients with no identified variant. High heterogeneity in several analyses suggests that further large cohort studies are needed.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1210\/clinem\/dgaf584","type":"journal-article","created":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T13:05:34Z","timestamp":1762002334000},"page":"580-590","source":"Crossref","is-referenced-by-count":7,"title":["Metastatic Disease in Phaeochromocytomas and Paragangliomas in Various Genotypes\u2014A Systematic Review and Meta-analysis"],"prefix":"10.1210","volume":"111","author":[{"given":"Louise Kirkegaard","family":"Svendsen","sequence":"first","affiliation":[{"name":"Department of Nephrology and Endocrinology, Copenhagen University Hospital: Rigshospitalet , Copenhagen 2100 ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9729-4767","authenticated-orcid":false,"given":"\u00c5se Krogh","family":"Rasmussen","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Endocrinology, Copenhagen University Hospital: Rigshospitalet , Copenhagen 2100 ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6075-0821","authenticated-orcid":false,"given":"Marianne Christina","family":"Klose","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Endocrinology, Copenhagen University Hospital: Rigshospitalet , Copenhagen 2100 ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4834-6724","authenticated-orcid":false,"given":"Jesper","family":"Krogh","sequence":"additional","affiliation":[{"name":"Clinic for Pituitary Disorders, Zealand University Hospital , K\u00f8ge 4600 ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5903-3355","authenticated-orcid":false,"given":"Ulla","family":"Feldt-Rasmussen","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Endocrinology, Copenhagen University Hospital: Rigshospitalet , Copenhagen 2100 ,","place":["Denmark"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2025,11,3]]},"reference":[{"issue":"3","key":"2026012101524783200_dgaf584-B1","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1007\/s12022-017-9484-5","article-title":"Update on adrenal tumours in 2017 world health organization (WHO) of endocrine tumours","volume":"28","author":"Lam","year":"2017","journal-title":"Endocr Pathol"},{"issue":"8","key":"2026012101524783200_dgaf584-B2","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.1097\/HJH.0000000000002438","article-title":"Genetics, diagnosis, management and future directions of research of phaeochromocytoma and paraganglioma: a position statement and consensus of the working group on endocrine hypertension of the European society of hypertension","volume":"38","author":"Lenders","year":"2020","journal-title":"J Hypertens"},{"issue":"2","key":"2026012101524783200_dgaf584-B3","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1210\/endrev\/bnab019","article-title":"Personalized management of pheochromocytoma and paraganglioma","volume":"43","author":"Nolting","year":"2022","journal-title":"Endocr Rev"},{"issue":"10","key":"2026012101524783200_dgaf584-B4","doi-asserted-by":"crossref","first-page":"2389","DOI":"10.3390\/cancers13102389","article-title":"Mutation profile of aggressive pheochromocytoma and paraganglioma with comparison of TCGA data","volume":"13","author":"Choi","year":"2021","journal-title":"Cancers (Basel)"},{"issue":"6","key":"2026012101524783200_dgaf584-B5","doi-asserted-by":"crossref","first-page":"2367","DOI":"10.1210\/jc.2018-01968","article-title":"Prognosis of malignant pheochromocytoma and paraganglioma (MAPP-prono study): a European network for the study of adrenal tumors retrospective study","volume":"104","author":"Hescot","year":"2019","journal-title":"J Clin Endocrinol Metab"},{"key":"2026012101524783200_dgaf584-B6","doi-asserted-by":"crossref","unstructured":"Svendsen LK, Rasmussen \u00c5K, Klose MC, Krogh J, Feldt-Rasmussen U. 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