Preferred Label : Multiple endocrine neoplasia type 1;
ICD-11 definition : 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.;
ICD-11 synonym : MEN1 - [multiple endocrine neoplasia type 1];
ICD-11 acronym : MEN1;
Origin ID : 1638765741;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
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.
