MEN1 syndrome | definition of MEN1 syndrome by Medical dictionary
Multiple endocrine neoplasia type 1 (redirected from MEN1 syndrome)
MEN 1 An inherited neoplasia syndrome characterised by: parathyroid hyperplasia (95%), pituitary adenoma (10%), pancreatic islet hyperplasia, adenoma or carcinoma, and/or adrenal cortical hyperplasia, adenoma and carcinoid tumours.
Mutation of MEN1 on chromosome 11.
Multiple endocrine neoplasia type 1 (MEN-1)
An inherited condition marked by multiple malignancies of the pituitary gland, parathyroid gland, and islet cells of the pancreas. About half of MEN-1 patients with pancreatic islet cell tumors will have gastrinomas, gastrin-producing tumors that lead to ulcer disease.
References in periodicals archive
These features are frequently seen not only in von Hippel-Lindau syndrome but also in sporadic pancreatic NETs and in association with MEN1 syndrome
Instead, hereditary MEN1 gene mutations give rise to MEN1 syndrome
, in which primary tumors occur in two or more endocrine glands.
In the case of the MEN1 gene, evidence is beginning to accumulate that suggests that its function is likely to be lost in some sporadic cancers of the same tissues affected in the hereditary MEN1 syndrome
While the underlying mechanism of this finding is largely unknown, this phenomenon is attributed to the status of pseudohypoxia resulting in HIF-1[alpha] increase in the setting of VHL disease, (15) since positivity for inhibin has not been observed in other clear cell NETs associated with MEN1 syndrome