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eRAM

encyclopedia of Rare Disease Annotation for Precision Medicine




Disease neuroepithelioma
Comorbidity C0027662|multiple endocrine neoplasia
Sentences 3
PubMedID- 25667631 Although insulinomas are often sporadic, they account for 10–30% of functioning pnets in patients with multiple endocrine neoplasia type 1. in addition, these tumors have been reported in patients with neurofibromatosis type 1 (7,8).
PubMedID- 20857521 For curative resection of functioning pnet associated with multiple endocrine neoplasia type 1 (men 1) which are usually multiple and sometimes numerous, resection surgery of the pancreas and/or the duodenum has to be performed based on localization by the sasi test.
PubMedID- 25565142 Biallelic inactivation of men1 encoding menin in pancreatic neuroendocrine tumors (pnets) associated with the multiple endocrine neoplasia type 1 (men1) syndrome is well established, but how menin loss/inactivation initiates tumorigenesis is not well understood.

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