Most patients with<unk>  are diagnosed in adulthood. Patients with<unk>  usually present with vague abdominal discomfort. Imaging with CT or MRI identifies a very large pancreas with one or more tumors. Biochemical testing shows marked hyperglucagonemia (hundreds-fold elevated). 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<unk> . Although the tumors commonly are indolent, death due to liver metastasis has been reported.
Mahvash disease
Dejerine–Sottas disease
Keshan disease
Yushō disease
Barakat syndrome
Satoyoshi syndrome
Sugarman syndrome
Nicolau–Balus syndrome