Radiosurgery for Catecholamine-Secreting Jugular Paragangliomas

Background Catecholamine-secreting jugular paragangliomas (JPs) represent a rare subset of head and neck paragangliomas that may manifest in hypertension, arrhythmia, or syncopal episodes. Upfront microsurgery is commonly utilized to attain tumor control and biochemical cure. However, hypertensive crises may complicate surgery and anesthetic management if appropriate adrenergic blockade is not achieved. Furthermore, subtotal resection to protect critical neurovascular structures may result in persistent catecholamine excess from viable residual tumor cells. Herein, we report our experience with stereotactic radiosurgery (SRS) in the primary and salvage management of localized, catecholamine-secreting JPs.

Setting Tertiary academic referral center.

Methods A retrospective review of adult patients with JP treated with SRS for catecholamine-secreting JP was performed. Posttreatment catecholamine levels, clinical outcomes, and tumor control are reported.

Results Of 85 patients with JPs treated with radiosurgery between 1990 and 2017, 3 (4%) harbored secreting tumors. One patient was excluded due to having metastatic disease. Patient 1 developed symptomatic arrhythmia necessitating pacemaker placement and was subsequently found to have a JP. Urine norepinephrine (NE) was elevated at 213 µg/24 hours. She underwent endovascular embolization and planned aggressive subtotal resection to reduce disease volume and protect lower cranial nerves. SRS was used to treat residual tumor (marginal dose 16 Gy, maximum dose 32 Gy, treatment volume 10,510 mm³). After treatment, catecholamine levels normalized and the cardiac arrhythmia resolved. The area of residual JP has not enlarged in size following SRS. Patient 2 developed catecholamine excess with elevated serum NE at 475 µg/24 hours. She underwent subtotal tumor resection with a postoperative nadir NE of 367 µg/24 hours. She developed catecholamine excess as a sign of tumor recurrence with peak serum NE of 639 µg/24 hours. She underwent SRS (marginal dose 15 Gy, maximum dose 30 Gy, treatment volume 11700 mm³) and over the ensuing 3 years catecholamine levels decreased to normal, and remained so over a 6-year period (serum NE 62 µg/24 hours at 10 years following SRS). Both patients have had durable tumor control (median follow-up: 89 months). The three additional published cases using SRS for treatment of secreting JP are reviewed.

Conclusion Radiosurgery can be utilized in the primary and salvage treatment of catecholamine-secreting JP, with durable tumor control and resolution of catecholamine excess. Further study of this rare subset of patients is warranted to corroborate these promising findings.