Endocrine Abstracts

Introduction: Paragangliomas are rare tumours that arise from paraganglia of neural crest origin. They have an incidence of approximately 1:300,000 and a third arise in the head and neck. A significant number of cervical paragangliomas are relatively asymptomatic, causing little more than a lump in the neck. The rest can cause cranial nerve palsies and infiltrate the skull base. Surgical treatment may cause the same deficits and, in some, risks more serious neurological deficits. The aim of this study is to review our experience in the management of these tumours.

Methods: This is a retrospective study involving cases of cervical paragangliomas, taken care of in our department, over a period of 10 years (2012-2021).

Results: A total of 6 cervical paragangliomas were identified in 5 patients. There were 3 women (60%) and two men (40%) with a mean age of 53.4 years (range: 32-68 years). There was not a family history for paragangliomas. A slow growing, painless neck mass was the main clinical presentation in all cases. There was no evidence of a functional tumor. Physical examination revealed a firm lateral cervical swelling. There were no cervical lymphadenopathy or nerve palsy in all cases. In preoperative imaging, neck ultrasound was performed for all patients. Computed tomography was performed in 4 cases and imagnetic resonance imaging in 3 cases. Of these 5 cases, 4 patients had carotid body paragangliomas, one of which is bilateral. Only one patient had vagal paraganglioma. We did not observe any malignant tumor. Secretory activity was studied in all cases an all patients had normal level of metanephrines. In our study, 3 patients were treated with surgery without postoperative complication in follow-up. One patient was treated with radiotherapy, while there was a patient who was not followed up.

Conclusions: Cervical paragangliomas are slow-growing tumours. Due to its localization near large vascular structures and cranial nerves, the surgical treatment is challenging. The surgery should be as conservative as possible to minimize the complications. In that aspect, preoperative embolization was mainly advised in large and hyper-vascularized tumors. However, in some parts of the world, radiotherapy (whether conventionally fractionated or as a single treatment from the ‘gamma knife’) or a watchful waiting policy is preferred.