Outcomes and Therapeutic Strategies for Head-and-Neck Paragangliomas Associated With Succinate Dehydrogenase Mutations

Anne-Claire Devouge, Hélène Lasolle, Charles Dupin, Julien Vergniol, Erwan De-Mones-Del Pujol, Juliette Abeillon, Sophie Giraud, Philippe Ceruse, Mélanie Gaudillière, Françoise Borson-Chazot, Gérald Raverot, Antoine Tabarin, Charlotte Lussey-Lepoutre, Magalie Haissaguerre
The Journal of Clinical Endocrinology & Metabolism, Volume 110, Issue 12, December 2025, Pages e4030–e4037
https://doi.org/10.1210/clinem/dgaf193

Abstract

Context

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.

Methods

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 < .001). UTC was more frequent in the JT than in other locations (39% vs 11%, P < .002).

Results

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 < .001).

Conclusions

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.

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