Endocrine Abstracts

Multiple endocrine neoplasia type 1 (MEN1) is a relatively rare autosomal dominantly inherited condition characterized by hyperplastic and neoplastic disorders of endocrine organs such as the parathyroid, anterior pituitary and gastroenteropancreatic endocrine tissues. Germline mutation of the causative gene, MEN1, which localizes to human chromosome 11q13 and encodes the 610 amino-acid nuclear protein menin, can be identified in most affected subjects. MEN1 gene mutation anal...

More than half of patients with multiple endocrine neoplasia type 1 (MEN1) develop gastroenteropancreatic neuroendocrine tumors (GEPNETs). Among GEPNETs in MEN1, insulinoma is the second common functioning tumor and often diagnosed by its pathognomonic clinical feature, i.e. hypoglycemia. Compared to other functioning and nonfunctioning GEPNETs in MEN1, insulinoma is known to develop in younger age. We have recently constructed a database of Japanese patients with MEN1 and her...