If the tumors are resected, they are found to be neuroendocrine tumors that usually express glucagon. In the tumor margin, pancreatic alpha cell hyperplasia is pervasive, numerous large islets composed of mostly alpha cells are evident, and multiple microadenomas and small neuroendocrine tumors often are present. The pancreatic neuroendocrine tumors are the main health concern for patients with Mahvash disease.