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Surgical Management of the Succinate Dehydrogenase–Associated Familial Paraganglioma Syndromes

Andrei Cocieru, MD; Pierre F. Saldinger, MD
Arch Surg. 2012;147(1):89-91. doi:10.1001/archsurg.2011.552.
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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.

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Graphic Jump Location

Figure 1. Computed tomographic scan of the abdomen of patient 1. The T indicates the para-aortic paraganglioma location; A, anterior, P, posterior; R, right; and L, left.

Place holder to copy figure label and caption
Graphic Jump Location

Figure 2. Computed tomographic scan of the abdomen of patient 2. The T indicates the retrocaval paraganglioma, located in the immediate vicinity of the right adrenal gland. A indicates anterior and P indicates posterior.




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