Diagnosis and management of multiple paragangliomas of the head and neck

Paragangliomas (PGs) are slowly growing, usually benign neoplasms. The aim of the study was to analyze the incidence, diagnostic and therapeutic management of patients with multiple paragangliomas of the head and neck. A retrospective review of the records of 84 patients with head and neck PGs, diagnosed and treated in our institution was performed for the years 1983–2013 to identify patients with multiple tumors. Fourteen (16.6 %) patients developed multiple PGs, synchronous or metachronous, within 4–21 years of follow-up. Clinical data of these patients were reviewed to evaluate the diagnosis, location, stage and management strategy. There was a total number of 37 tumors in 14 patients. There were 20/37 (54.0 %) carotid PGs, 9/37 (24.3 %) jugular PGs and 8/37 (21.7 %) vagal PGs. Carotid PGs were observed in 12/14 (86 %) patients and in 8/14 (57 %) cases bilateral tumors occurred. Vagal PGs developed in 7/14 (50 %) patients and bilateral tumors were found in 1/14 (7 %) case. Jugular PGs occurred in 9/14 (64 %) patients. There were 30 synchronous tumors and seven metachronous PGs diagnosed 2–18 years after removal of the first tumor. Single metachronous mediastinal PG occurred. All patients had at least one tumor removed, with histopathological confirmation of the diagnosis. One patient had positive history of familial PGs. Carotid PGs are most common multiple paragangliomas. Radiological survey of the head and neck is required to detect multicentric tumors. Metachronous mediastinal and abdominal tumors may occur. Regular, prolonged follow-up is essential to identify metachronous PGs and possible postoperative gradual ICA occlusion.