Head and neck paragangliomas. An interdisciplinary challenge

Current treatment strategies for head and neck paragangliomas are moving away from radical resection and toward surgical tumor reduction, in order to preserve function and reduce morbidity. Radiotherapy modalities are alternative primary treatment options. A PubMed search of the relevant literature on genetics and treatment of head and neck paragangliomas was conducted. The rapid progress made in genetic research was mainly triggered by two factors: firstly, the establishment of central registries for paraganglioma patients and secondly, the availability of next-generation sequencing methods. Exome sequencing and a gene-panel sequencing approach have already been successfully applied to paraganglioma syndromes. The latter method in particular is rapid and cost-effective, and may soon replace complex genotyping algorithms. The literature provides good evidence that diversified modern treatment options are available to realize individual treatment concepts for almost all paraganglioma manifestations. Generally, small and symptomatic tumors should be completely resected, particularly in younger patients. Considering the patient's age, symptoms, morbidity risk, and comorbidities, larger tumors should be surgically treated in a function-preserving manner. In these cases, primary radiotherapy is an equivalent alternative option. A "wait and scan" strategy is possible in selected cases. The potential morbidity of surgical treatment must be weighed against the expectable quality of life. Comprehensive consultation with the patient about possible treatment modalities is mandatory. Treatment decision making should involve a multidisciplinary team of experts.