Head and Neck Paragangliomas

Head and neck paragangliomas are highly vascularized neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. These tumors may occur either sporadically or in the context of a hereditary familial tumor syndrome, and multifocal presentations are observed, particularly in hereditary cases. Hereditary paragangliomas are mostly caused by mutations in the succinate dehydrogenase complex genes. Early imaging, with ultrasonography of the neck and magnetic resonance imaging (MRI) of the skull base, is essential for localizing and assessing the extent of the tumor, as well as for precise planning of the treatment approach. Views regarding the treatment of choice are generally moving away from radical resection toward surgical tumor reduction in order to preserve function and reduce morbidity. Radiotherapy modalities are alternative primary treatment options, depending on the individual situation (e.g., in relation to age, comorbidity, multifocal lesions, and risk of injury to the cranial nerves). Observation is an option in selected patients. The potential morbidity of surgical treatment must be weighed against the expectable quality of life, and comprehensive consultation with the patient about the possible treatment modalities is mandatory. Treatment decision-making should involve a multidisciplinary team of experts in the fields of nuclear medicine, genetics, pathology, radiology, radio-oncology, and surgery.