Outcomes and Therapeutic Strategies for Head-and-neck Paragangliomas Associated With Succinate Dehydrogenase...

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.

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).

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).

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.