Article
[Inherited tumor syndromes of gastroenteropancreatic and thoracic neuroendocrine neoplasms].
Annales de pathologie - 1 Apr 2020
Couvelard Anne, Scoazec Jean-Yves
Abstract excerpt
About 5% of gastroenteropancreatic and thoracic neuroendocrine neoplasms (NENs) arise in the context of an inherited tumour syndrome. The two most frequent syndromes are: multiple endocrine neoplasia type 1 (MEN1), associated with a large spectrum of endocrine and non endocrine tumours, including duodenopancreatic, thymic and bronchial NENs, and the von Hippel-Lindau syndrome VHL, associated with pancreatic NENs....
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