{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T01:41:55Z","timestamp":1791942115080,"version":"4.3.4"},"reference-count":32,"publisher":"The Endocrine Society","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,10,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Context: Pheochromocytomas and paragangliomas may be malignant either at presentation or during recurrence, but the clinical course of malignant tumors is unpredictable.<\/jats:p>\n                  <jats:p>Objective: The objective was to analyze survival according to clinical characteristics at diagnosis of malignancy and the presence or absence of SDHB mutations.<\/jats:p>\n                  <jats:p>Design: This was a retrospective cohort study.<\/jats:p>\n                  <jats:p>Setting and Participants: A total of 54 patients with malignant tumors were included. Malignancy was scored according to the presence of metastases or histologically documented lymph node invasion.<\/jats:p>\n                  <jats:p>Main Outcome Measures: The main outcome was the specific survival after the diagnosis of the first metastasis.<\/jats:p>\n                  <jats:p>Results: Germline mutations were identified in SDHB (n = 23, including 21 patients with apparent sporadic tumors) and VHL (n = 1) genes, and two patients had neurofibromatosis 1. Patients were followed up from the diagnosis of primary tumor and from the diagnosis of the first metastasis to the present or to death with medians of 79 [interquartile range (IQR) 24; 190] and 39 [IQR 14; 94] months, respectively. The 5-yr probability of survival after the diagnosis of the first metastasis was 0.55 (95% confidence interval 0.39\u20130.69). Patients with SDHB mutations were younger, more frequently had extra-adrenal tumors, and had a shorter metanephrine excretion doubling time. The presence of SDHB mutations was significantly and independently associated with mortality (relative risk 2.7; 95% confidence interval 1.2, 6.4; P = 0.021).<\/jats:p>\n                  <jats:p>Conclusion: SDHB mutations, frequent in patients with malignant pheochromocytomas or paragangliomas, are associated with shorter survival. Therefore, SDHB genetic testing may be of prognostic value for such patients, even those with an apparent sporadic and\/or benign presentation at diagnosis.<\/jats:p>","DOI":"10.1210\/jc.2007-0709","type":"journal-article","created":{"date-parts":[[2007,7,24]],"date-time":"2007-07-24T21:04:15Z","timestamp":1185311055000},"page":"3822-3828","source":"Crossref","is-referenced-by-count":401,"title":["Succinate Dehydrogenase B Gene Mutations Predict Survival in Patients with Malignant Pheochromocytomas or Paragangliomas"],"prefix":"10.1210","volume":"92","author":[{"given":"Laurence","family":"Amar","sequence":"first","affiliation":[{"name":"Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale U772 (L.A., N.B., X.J., A.-P.G.-R., P.-F.P.), Colle\u0300ge de France, 75231 Paris, France"},{"name":"Department of Genetics (L.A., N.B., A.-P.G.-R., X.J.), Ho\u0302pital Europe\u0301en Georges Pompidou, 75908 Paris cedex 15, France"},{"name":"Departments of Hypertension Unit (L.A., P.-F.P.), Ho\u0302pital Europe\u0301en Georges Pompidou, 75908 Paris cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Baudin","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine and Endocrine Tumors (E.B., M.S.), Institut Gustave Roussy, 94805 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nelly","family":"Burnichon","sequence":"additional","affiliation":[{"name":"Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale U772 (L.A., N.B., X.J., A.-P.G.-R., P.-F.P.), Colle\u0300ge de France, 75231 Paris, France"},{"name":"Department of Genetics (L.A., N.B., A.-P.G.-R., X.J.), Ho\u0302pital Europe\u0301en Georges Pompidou, 75908 Paris cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Se\u0301verine","family":"Peyrard","sequence":"additional","affiliation":[{"name":"Clinical Investigation Centre 9201 (S.P.), Ho\u0302pital 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Sante\u0301 et de la Recherche Me\u0301dicale U-567 (J.B., X.B.), Faculte\u0301 de Me\u0301decine Rene\u0301 Descartes, Universite\u0301 Paris 5, 75270 Paris cedex, France"},{"name":"Departments of Endocrinology and Radiology (S.S., J.B., X.B.), Ho\u0302pital Cochin, 75679 Paris cedex 14, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Schlumberger","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine and Endocrine Tumors (E.B., M.S.), Institut Gustave Roussy, 94805 Villejuif, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier","family":"Jeunemaitre","sequence":"additional","affiliation":[{"name":"Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale U772 (L.A., N.B., X.J., A.-P.G.-R., P.-F.P.), Colle\u0300ge de France, 75231 Paris, France"},{"name":"Department of Genetics (L.A., N.B., A.-P.G.-R., X.J.), Ho\u0302pital Europe\u0301en Georges Pompidou, 75908 Paris cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Paule","family":"Gimenez-Roqueplo","sequence":"additional","affiliation":[{"name":"Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale U772 (L.A., N.B., X.J., A.-P.G.-R., P.-F.P.), Colle\u0300ge de France, 75231 Paris, France"},{"name":"Department of Genetics (L.A., N.B., A.-P.G.-R., X.J.), Ho\u0302pital Europe\u0301en Georges Pompidou, 75908 Paris cedex 15, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre-Franc\u0327ois","family":"Plouin","sequence":"additional","affiliation":[{"name":"Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale U772 (L.A., N.B., X.J., A.-P.G.-R., P.-F.P.), Colle\u0300ge de France, 75231 Paris, France"},{"name":"Departments of Hypertension Unit (L.A., P.-F.P.), Ho\u0302pital Europe\u0301en Georges Pompidou, 75908 Paris cedex 15, 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