The Surgery of Glomus Jugulare Tumors: a Reasoned Challenge

Aim: To delineate the philosophy of our School in the management of jugular paraganglioma (JP), based on the experience of 88 cases treated over 25 years by the same surgeonResults: Seven prerequisites have been identified before any decision is taken: 1/a complete analysis of cranial nerves (CN) functions (CN6-12); 2/a thorough multidisciplinary evaluation of the patient’s general condition; 3/a complete imaging work-up (MRI, 4D angio-MR, CT scan); 4/ the availability of a high-level technical and medical support. Neurosurgeon, speech therapist, neuroradiologist, geneticist, endocrinologist are necessary to the patient’s evaluation and pre and postoperative management; 5/ a dedicated inform consent including information about surgery and embolization; 6/ a multidisciplinary meeting to decide which management is to be used: wait and scan, surgery or radiotherapy; 7/a genetic work-up including clinical evaluation of the family and blood samples for mutation research. Surgery is preferred in case of patients <50 yrs, in genetically induced lesions, in rapidly growing monitored tumors, in case of cranial nerve palsy (7, 9-12) and when radiation therapy was unsuccessful.Conclusion: Surgery of JP remains a challenge. But it must be reasoned, as the result of a planned multidisciplinary clinical and instrumental path. The tumor removal is highly demanding for the otologist. Paragangliomas are generally difficult to manage but JP are the most complex because of: a/the lack of clear tumor limits in the petrous bone; b/ the vital arterial and venous structures involvement; c/the intracranial penetration through the jugular foramen; d/the risk of life-threatening CN6-12 compromises; e/ the difficulty for the radiologist to penetrate in all the feeding vessels. For all these reasons, exclusive radiotherapy is taking an increasing role in the management of some JPs since it may spare cranial nerves functions.