{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T01:43:58Z","timestamp":1791942238093,"version":"4.3.4"},"reference-count":44,"publisher":"The Endocrine Society","issue":"10","license":[{"start":{"date-parts":[[2022,8,10]],"date-time":"2022-08-10T00:00:00Z","timestamp":1660089600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"name":"Groupe des tumeurs endocrines"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,9,28]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Context<\/jats:title>\n                  <jats:p>Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that are frequently associated with succinate dehydrogenase (SDH) germline mutations. When mutated, SDH losses its function, thus leading to succinate accumulation.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>In this study, we evaluated serum succinate levels as a new metabolic biomarker in SDHx-related carriers.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Retrospective monocentric study of 88 PPGL patients (43 sporadic, 35 SDHB, 10 SDHA\/C\/D), 17 tumor-free familial asymptomatic carriers (13 SDHB, 4 SDHC\/D), and 60 healthy controls. Clinical, biological, and imaging data were reviewed. Serum succinate levels (n\u2005=\u2005280) were quantified by an ultra-performance liquid chromatography coupled to a tandem mass spectrometry method and correlated to SDHx mutational status, disease extension, and other biological biomarkers.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Serum succinate levels\u2005&amp;gt;\u20057 \u03bcM allowed identification of tumor-free asymptomatic SDHB-mutated cases compared to a healthy control group (100% specificity; 85% sensitivity). At PPGL diagnosis, SDHB-mutated patients had a significantly increased median succinate level (14 \u03bcM) compared to sporadic patients (8 \u03bcM) (P\u2005&amp;lt;\u20050.01). Metastatic disease extension was correlated to serum succinate levels (r\u2005=\u20050.81). In the SDHB group, patients displaying highest tumor burdens showed significant increased succinate levels compared to the sporadic group (P\u2005&amp;lt;\u20050.0001).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>In this pilot study, we showed that serum succinate level is an oncometabolic biomarker that should be useful to identify SDHB-related carriers. Succinate levels are also a marker of metabolic tumor burden in patients with a metastatic PPGL and a potential marker of treatment response and follow-up.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1210\/clinem\/dgac474","type":"journal-article","created":{"date-parts":[[2022,8,10]],"date-time":"2022-08-10T23:41:26Z","timestamp":1660174886000},"page":"2801-2810","source":"Crossref","is-referenced-by-count":24,"title":["Succinate: A Serum Biomarker of <i>SDHB<\/i>-Mutated Paragangliomas and Pheochromocytomas"],"prefix":"10.1210","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0311-8031","authenticated-orcid":false,"given":"Constance","family":"Lamy","sequence":"first","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, Inserm UMR1030, Molecular Radiotherapy and Therapeutic Innovation , Villejuif , France"},{"name":"Gustave Roussy , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hubert","family":"Tissot","sequence":"additional","affiliation":[{"name":"Gustave Roussy, Department of Nuclear Medicine , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthieu","family":"Faron","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, UVSQ, Inserm, CESP , Villejuif , France"},{"name":"Gustave Roussy, Department of Digestive Surgery , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2198-3582","authenticated-orcid":false,"given":"Eric","family":"Baudin","sequence":"additional","affiliation":[{"name":"Gustave Roussy, Department of Endocrine Oncology , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Livia","family":"Lamartina","sequence":"additional","affiliation":[{"name":"Gustave Roussy, Department of Endocrine Oncology , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Pradon","sequence":"additional","affiliation":[{"name":"Gustave Roussy, Department of Medical Biology and Pathology , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abir","family":"Al Ghuzlan","sequence":"additional","affiliation":[{"name":"Gustave Roussy, Department of Medical Biology and Pathology , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Leboulleux","sequence":"additional","affiliation":[{"name":"Gustave Roussy, Department of Nuclear Medicine , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Luc","family":"Perfettini","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, Inserm UMR1030, Molecular Radiotherapy and Therapeutic Innovation , Villejuif , France"},{"name":"Gustave Roussy , Villejuif , France"},{"name":"Department of Biomedical Sciences, University of the Pacific, Arthur A. Dugoni School of Dentistry , 155 Fifth Street, San Francisco, CA 94103 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelo","family":"Paci","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, Inserm UMR1030, Molecular Radiotherapy and Therapeutic Innovation , Villejuif , France"},{"name":"Gustave Roussy, Department of Medical Biology and Pathology , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Hadoux","sequence":"additional","affiliation":[{"name":"Gustave Roussy, Department of Endocrine Oncology , Villejuif , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6333-3636","authenticated-orcid":false,"given":"Sophie","family":"Broutin","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Paris-Saclay, Gustave Roussy, Inserm UMR1030, Molecular Radiotherapy and Therapeutic Innovation , Villejuif , 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