Management of complex tympanojugular paragangliomas including endovascular intervention.

Objectives/hypothesis: To identify complex cases of tympanojugular paraganglioma (TJP) and to analyze their management and surgical outcome. To present strategy and guidelines on how to deal with the perioperative complicating factors.

Study Design: Retrospective analysis.

Methods: We retrospectively studied 212 TJP patients to identify the complex cases and analyzed their management and surgical outcome. The criteria for complex TJP are: 1) very large size; 2) large intradural extension (IDE); 3) extension to the cavernous sinus, foramen magnum, and clivus; 4) significant involvement of the internal carotid artery (ICA); 5) a solitary ICA on the lesion side; 6) involvement of the vertebral artery; 7) dominant or unilateral sigmoid sinus on the lesion side; 8) bilateral or multiple paragangliomas; and 9) recurrence.

Results: Sixty patients were classified as complex. Forty-five patients had tumors with IDE. Twenty patients with tumor involving ICA underwent preoperative endovascular intervention. Two patients had a solitary ICA and two others unilateral sigmoid sinus on the lesion side. Thirteen patients had multicentric tumors, and three patients had bilateral ones. The main procedure was the infratemporal fossa approach type A. Total removal was achieved in 46 patients, and five of them experienced recurrence.

Conclusions: It is essential to carefully investigate the hemodynamics of the brain in planning surgery. A proper preoperative endovascular intervention facilitates gross total tumor removal. In bilateral paragangliomas, lower cranial nerves' function should be preserved at least unilaterally. Staged removal is recommended for a tumor with a large intradural component.