{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,12]],"date-time":"2026-11-12T13:38:25Z","timestamp":1794490705347,"version":"4.9.1"},"reference-count":42,"publisher":"The Endocrine Society","issue":"5","license":[{"start":{"date-parts":[[2022,1,17]],"date-time":"2022-01-17T00:00:00Z","timestamp":1642377600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Hillcrest Foundation","id":[{"id":"https:\/\/ror.org\/02g53td78","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,4,19]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Context<\/jats:title>\n                    <jats:p>Carriers of succinate dehydrogenase type B (SDHB) pathogenic variants (PVs) are at risk of pheochromocytoma and paraganglioma (PPGL) from a young age. It is widely recommended carriers enter a surveillance program to detect tumors, but there are limited studies addressing outcomes of surveillance protocols for SDHB PV carriers.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The purpose of this study was to describe surveillance-detected (s-d) tumors in SDHB PV carriers enrolled in a surveillance program and to compare their outcomes to probands.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This was a multicenter study of SDHB PV carriers with at least 1 surveillance episode (clinical, biochemical, imaging) in Australian genetics clinics. Data were collected by both retrospective and ongoing prospective follow-up. Median duration of follow-up was 6.0 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>181 SDHB PV carriers (33 probands and 148 nonprobands) were assessed. Tumors were detected in 20% of nonprobands undergoing surveillance (age range 9-76 years). Estimated 10-year metastasis-free survival was 66% for probands and 84% for nonprobands with s-d tumors (P\u2005=\u2005.027). S-d tumors were smaller than those in probands (median 27 mm vs 45 mm respectively, P\u2005=\u2005.001). Tumor size \u226540 mm was associated with progression to metastatic disease (OR 16.9, 95% CI 2.3-187.9, P\u2005=\u2005.001). Patients with s-d tumors had lower mortality compared to probands: 10-year overall survival was 79% for probands and 100% for nonprobands (P\u2005=\u2005.029).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>SDHB carriers with s-d tumors had smaller tumors, reduced risk of metastatic disease, and lower mortality than probands. Our results suggest that SDHB PV carriers should undertake surveillance to improve clinical outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1210\/clinem\/dgac019","type":"journal-article","created":{"date-parts":[[2022,1,17]],"date-time":"2022-01-17T11:14:32Z","timestamp":1642418072000},"page":"e1907-e1916","source":"Crossref","is-referenced-by-count":35,"title":["Surveillance Improves Outcomes for Carriers of\n                    <i>SDHB<\/i>\n                    Pathogenic Variants: A Multicenter Study"],"prefix":"10.1210","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4084-9239","authenticated-orcid":false,"given":"Dahlia F","family":"Davidoff","sequence":"first","affiliation":[{"name":"Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, NSW, Australia"},{"name":"Faculty of Medicine and Health, University of Sydney, Sydney, Australia"},{"name":"Department of Endocrinology, Royal North Shore Hospital, St Leonards, NSW, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana E","family":"Benn","sequence":"additional","affiliation":[{"name":"Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, NSW, Australia"},{"name":"Faculty of Medicine and Health, University of Sydney, Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Field","sequence":"additional","affiliation":[{"name":"NSLHD Familial Cancer Service, Department of Cancer Services, Royal North Shore Hospital, St Leonards, NSW, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"Crook","sequence":"additional","affiliation":[{"name":"NSLHD Familial Cancer Service, Department of Cancer Services, Royal North Shore Hospital, St Leonards, NSW, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruce G","family":"Robinson","sequence":"additional","affiliation":[{"name":"Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, NSW, Australia"},{"name":"Faculty of Medicine and Health, University of Sydney, Sydney, Australia"},{"name":"Department of Endocrinology, Royal North Shore Hospital, St Leonards, NSW, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine","family":"Tucker","sequence":"additional","affiliation":[{"name":"Hereditary Cancer Centre, Prince of Wales Hospital, Randwick, NSW, Australia"},{"name":"Prince of Wales Clinical School, UNSW Medicine, Kensington, NSW, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard","family":"De Abreu Lourenco","sequence":"additional","affiliation":[{"name":"Centre for Health Economics Research and Evaluation, University of Technology Sydney, Haymarket, Sydney, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John R","family":"Burgess","sequence":"additional","affiliation":[{"name":"Department of 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