Familial paraganglioma of head and neck

Paragangliomas are tumors originated from non-chromaffin paranganglia cells derived from the neural crest. These highly vascular tumors are located in the region which extends from skull base to arcus aorta. Head and neck paragangliomas have rare occurence constituting 0.012% of all the body tumors and 0.6% of head and neck tumors. Head and neck paragangliomas occur in sporadic and familial forms. The ratio of familial head and neck paragangliomas is about 10%. Familial form has a characteristic of autosomal dominant inheritance. In this article, four cases having first and second degree of affinity that we followed for paraganglioma diagnosis are presented.