Surgical management of the succinate dehydrogenase-associated familial paraganglioma syndromes.

Overview

Paragangliomas are rare neuroendocrine tumors arising from the neural crest cells in the extra-adrenal location. Paragangliomas can be sporadic or associated with a range of endocrine and genetic syndromes in 25% to 30% of all cases. Specifically, succinate dehydrogenase gene mutations are involved in the development of paraganglioma syndromes type 1 through type 4. In this article, we will describe 2 cases of succinate dehydrogenase-associated familial paraganglioma syndrome and provide a review of the existing literature on the condition's etiologic factors, diagnosis, and management.