Head and neck paragangliomas: what does the pathologist need to know?

Paragangliomas can occur in a variety of anatomic locations in the head and neck region and can create diagnostic challenges for practicing pathologists. The most recent data suggest that at least 30–40% of paragangliomas are associated with inherited disease. Occasional VHL-, TMEM127-, and SDHA-related head and neck paragangliomas have been described; however, the bulk of hereditary disease in the head and neck paraganglioma is associated with SDHD, SDHC, SDHB, and SDHAF2 mutations. While the distinction of paragangliomas from other head and neck neoplasms is very important, the clinical responsibility of surgical pathologists has evolved and also includes the integration of SDHB immunohistochemistry into the routine pathology practice. In this article, we highlight an approach to clinicopathological diagnosis of head and neck paragangliomas along with a comprehensive discussion on genotype–biochemical profile correlation and synoptic report approach in paragangliomas.