Author(s)
Lindsay Scott Moore MD
Andrew Prince BS
Harishanker Jeyarajan MD
Rupert Obholzer MD
Michael Gleeson MD
Affiliation(s)
University of Alabama
Abstract:
Educational Objective: At the conclusion of this presentation, the participants should be able to discuss the issues surrounding management of the familial paraganglioma patient, as well as various primary treatment options, outcomes, and deficits. Objectives: Head and neck paragangliomas are slowly growing neuroendocrine tumors that are benign in over 90% of cases. However, 30-40% of patients have the familial form of paraganglioma. Management of familial patients with more complex, often bilateral and multiple tumors, is significantly more difficult than sporadic cases. Guidelines for management of familial paragangliomas requires is limited and further investigation and is required to direct treatment and optimize outcomes. Study Design: An institutional review board approved, retrospective review of a single surgeon’s database of paragangliomas was performed. Methods: Patients with proven genetic mutations or multicentric tumors were selected to create a cohort of complex, familial paragangliomas. Primary treatment (two different modalities in 23 patients with bilateral tumors), subsequent treatment needed, and resulting deficits were explored. Results: Of 266 patients with paragangliomas, 69 patients were included. Sixty-one of these patients had proven genetic mutations, 43 patients had bilateral tumors, and 56 patients had multiple tumors. There were 159 total tumors among the 69 patients, with a mean of 2.33 tumors per patient. Observation was the primary treatment for 35 cases. Fifteen (42.8%) eventually needed surgical intervention and 5 (14.2%) needed radiation therapy. Radiation therapy was the primary treatment in 14 cases and none failed treatment. Surgery was primary treatment in 43 cases and 7 (16.3%) required additional adjuvant therapy. 18 patients had impaired vocal function, 16 had hearing deficits, 3 had swallowing dysfunction, and 5 had first bite syndrome. Conclusions: Careful selection of a treatment regimen for familial paragangliomas is necessary to optimize outcomes and requires case-specific considerations.