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Although there are indicators to assess metastatic risks, none of them predict metastasis reliably. Therefore, we aimed to develop and validate a scoring system using clinical, genetic, and biochemical risk factors to preoperatively predict the metastatic risk of PPGL.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In the cross\u2010sectional cohort (<jats:italic>n<\/jats:italic>\u00a0=\u00a0180), clinical, genetic, and biochemical risk factors for metastasis were identified using multivariate logistic regression analysis, and a novel scoring system was developed. The scoring system was validated and compared with the age, size of tumor, extra\u2010adrenal location, and secretory type (ASES) score in the longitudinal cohort (<jats:italic>n<\/jats:italic>\u00a0=\u00a0114).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In the cross\u2010sectional cohort, pseudohypoxia group\u2010related gene variants (<jats:italic>SDHB<\/jats:italic>, <jats:italic>SDHD<\/jats:italic>, or <jats:italic>VHL<\/jats:italic>), methoxytyramine &gt;0.16\u00a0nmol\/L, and tumor size &gt;6.0\u00a0cm were independently associated with metastasis after multivariate logistic regression. Using them, the gene variant, methoxytyramine, and size of tumor (GMS) score were developed. In the longitudinal cohort, Harrell's concordance index of the GMS score (0.873, 95% confidence interval [CI]: 0.738\u20130.941) was higher than that of the ASES score (0.713, 95% CI: 0.567\u20130.814, <jats:italic>p<\/jats:italic>\u00a0=\u00a00.007). In the longitudinal cohort, a GMS score \u22652 was significantly associated with a higher risk of metastasis (hazard ratio\u00a0=\u00a025.07, 95% CI: 5.65\u2013111.20). A GMS score \u22652 (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a00.001), but not ASES score \u22652 (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.090), was associated with shorter progression\u2010free survival.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The GMS scoring system, which integrates gene variant, methoxytyramine level, and tumor size, provides a valuable preoperative approach to assess metastatic risk in PPGL.<\/jats:p><\/jats:sec>","DOI":"10.1111\/joim.13791","type":"journal-article","created":{"date-parts":[[2024,4,25]],"date-time":"2024-04-25T06:15:18Z","timestamp":1714025718000},"page":"68-79","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Preoperative prediction of metastatic pheochromocytoma and paraganglioma using clinical, genetic, and biochemical markers: A cohort study"],"prefix":"10.1111","volume":"296","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5719-1539","authenticated-orcid":false,"given":"Seung Shin","family":"Park","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Seoul National University College of Medicine  Seoul Republic of Korea"},{"name":"Department of Internal Medicine Seoul National University Hospital  Seoul Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chang Ho","family":"Ahn","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Bundang Hospital  Seongnam Republic of Korea"},{"name":"Lunit  Seoul Republic of Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seunghoo","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine Asan Medical Center University of Ulsan College of Medicine  Seoul Republic of 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