A novel hyperplasia-neoplasia sequence in pancreatic endocrine tumors: Glucagon cell adenomatosis

Introduction: Glucagon cell adenomatosis (GCA) was recently recognized by us as a multifocal neoplastic disease of the endocrine pancreas unrelated to MEN1. Multiple micro- and a few macrotumors are found on the background of a hyperplasia of glucagon cells. The disease may cause unspecific abdominal symptoms and only rarely a glucagonoma syndrome. We aimed to investigate the genetic alterations of GCGR gene in patients with GCA. Material and methods: FFPE pancreatic tissues from six patients showing multiple microadenomas and partly macroadenomas of glucagon cells were macro- or microdissected. Following DNA ex…