{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T01:41:53Z","timestamp":1791942113711,"version":"4.3.4"},"reference-count":41,"publisher":"The Endocrine Society","issue":"12","license":[{"start":{"date-parts":[[2025,4,21]],"date-time":"2025-04-21T00:00:00Z","timestamp":1745193600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,11,18]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Context<\/jats:title>\n                    <jats:p>Natural history and optimal therapeutic strategies of patients with head-and-neck paragangliomas (HNPGL) associated with germline mutations in succinate dehydrogenase genes (SDHx) are barely known. This study aims to describe the outcome of these patients depending on selected strategies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed the outcome of 65 SDHx-mutated patients presenting 108 HNPGL mostly located in the carotid (57%) and jugulotympanic (JT) (21.5%) areas. One hundred five HNPGLs (97%) were nonsecreting and nonmetastatic, with multiple tumors observed in 40 patients (62%). HNPGLs were initially managed by surgery for 56 (52%), monitoring for 31 (29%), and radiotherapy for 21 (19%). Unsuccessful tumor control (UTC) was defined as a tumor volume increase or a need to change therapeutic strategy. During a 7-year median follow-up period, 18 UTCs (17%) were observed in 17 patients. Among operated HNPGLs, 13 (23%) had an UTC, compared with 1 (5%) among the irradiated HNPGL and 4 (13%) among monitored HNPGLs. The incidence of UTC was significantly increased in HNPGL treated by incomplete surgical resection compared to HNPGL treated by complete surgery (50% vs 0%, P &amp;lt; .001). UTC was more frequent in the JT than in other locations (39% vs 11%, P &amp;lt; .002).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Posttherapeutic complications were observed in 34 patients (55%), mainly neurological (73%) or vascular (15%), with a higher incidence after surgery than after irradiation (66% vs 14%, P &amp;lt; .001).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Most SDHx patients with monitored HNPGLs had a stable disease confirming the interest of initial time for observation before deciding to treat or not, particularly in asymptomatic patients.<\/jats:p>\n                  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