[Inherited tumor syndromes of gastroenteropancreatic and thoracic neuroendocrine neoplasms].

doi:10.1016/j.annpat.2020.01.002

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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....

Topics

Duodenal Neoplasms
Genetic Predisposition to Disease
Humans
Intestinal Neoplasms
Intestines
Multiple Endocrine Neoplasia
Multiple Endocrine Neoplasia Type 1
Neoplastic Syndromes, Hereditary
Neuroendocrine Tumors
Pancreas
Pancreatic Neoplasms
Stomach Neoplasms
Thoracic Neoplasms
Thorax
Tuberous Sclerosis
von Hippel-Lindau Disease

Source

Open in PubMed

Identifiers

PMID: 32035641
DOI: 10.1016/j.annpat.2020.01.002

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