/somatostatinoma

somatostatinoma

Rare Disease

MONDO:0006976

Also Known As

Delta cell tumorDelta cell tumoursomatostatin cell neoplasmsomatostatin cell tumorsomatostatin cell tumoursomatostatin producing tumorsomatostatin producing tumoursomatostatin-producing NETsomatostatin-producing neuroendocrine tumorsomatostatin-producing neuroendocrine tumoursomatostatin-producing tumorsomatostatin-producing tumoursomatostatinomatumor of Delta cellstumor of the Delta cellstumour of Delta cellstumour of the Delta cellsSomatomedin-secreting carcinoidampullary somatostatinomacarcinoid somatostatinomamalignant islet cell tumormalignant islet cell tumoursomatostatin-secreting pancreatic neoplasm

Definition

A rare, usually malignant neuroendocrine tumor arizing from delta cells. This neoplasm produces large amounts of somatostatin, which may result in a syndrome characterized by diarrhea, steatorrhea, weight loss, and gastric hyposecretion. Sixty percent are found in the pancreas and 40% in the duodenum or jejunum. The peak incidence occurs between 40 and 60 years of age; women are affected more than men by 2:1.