16h40 Carotid body paraganglioma – presentation, management and pitfalls

Keywords: paraganglioma, carotid body, interdisciplinary management.

Objectives: paragangliomas are a group of neuroendocrine tumors arising from the paraganglia. They are rare, accounting for 0,6% of the tumors in the cervicofacial region. Although generally benign, 6- 19% of all head and neck paragangliomas develop metastases. Risk factors include chronic or intermittent hypoxemia and genetic predisposition. Due to their close proximity to vital structures, adequate recognition and treatment are of utmost importance to avoid unfavorable outcomes.

Material and Methods: in this presentation, key concepts are illustrated by aid of our own patient data.

Results:  the clinical presentation of carotid body tumors consists of a cervical mass, dysphonia, tinnitus and headache. Symptoms related to cranial nerve compression and catecholamine release can sometimes be seen as well. The diagnosis is based on clinical examination and imaging (CT, MRI, arteriography). Obtaining a fine- needle aspiration biopsy is contraindicated due to possible bradycardia. Treatment options are conservative, surgical resection with or without preoperative embolization, radiotherapy or a combination of these options. An initial annual follow-up is recommended for the first 3 years. The interval can be lengthened afterwards to every other year for six years and every 3 years for the remainder.

Conclusion: carotid body paragangliomas require a specific approach. Interdisciplinary handling by a team of maxillofacial and vascular surgeons improves the outcome. Physicians should be aware of possible complications. Novel innovative techniques arise like treatment of locally unresectable somatostatin receptor (SSTR) imaging-positive paragangliomas with SSTR analogs. Genetic testing and counseling are recommended since germline succinate dehydrogenase mutations can indicate risk of multifocal and metastatic disease..