Catalog

Multiple endocrine neoplasia type 1

No MMS code

Link to WHO Definition

Multiple endocrine neoplasia type 1 – http://id.who.int/icd/entity/1638765741

Multiple Endocrine Neoplasia type 1 (MEN1) is a rare hereditary cancer syndrome marked mainly by tumours of the parathyroids, endocrine pancreas and anterior pituitary, and characterised by a very high penetrance and an equal sex distribution. Two different forms, sporadic and familial, have been described. The sporadic form presents with two of the three principal MEN1-related endocrine tumours (parathyroid adenomas, entero-pancreatic tumours and pituitary tumours) within a single patient, while the familial form consists of a MEN1 case with at least one first-degree relative showing one of the characteristic endocrine tumours.