somatostatinoma
Pronunciation: so-MAT-oh-stat-in-oh-muh
A rare functional neuroendocrine tumor secreting somatostatin, often causing diabetes and gallbladder dysfunction.
Full Definition
Somatostatinomas are extremely rare functional neuroendocrine tumors that secrete excessive somatostatin hormone. These tumors can arise in the pancreas or duodenum, with duodenal tumors often associated with neurofibromatosis type 1. The classic somatostatinoma syndrome includes diabetes mellitus, cholelithiasis, and steatorrhea, though this complete triad is present in less than 10% of patients. Most somatostatinomas are large and malignant at presentation. Diagnosis requires elevated plasma somatostatin levels in conjunction with appropriate clinical findings. Treatment involves surgical resection when feasible, though the rarity of these tumors limits treatment experience and outcomes data.
Usage
Usage note: Extremely rare; often associated with NF1 when duodenal in location.
In Context
- "The duodenal somatostatinoma was associated with the patient's known neurofibromatosis type 1." — Genetic medicine consultation
- "Plasma somatostatin levels of 850 pg/mL supported the rare diagnosis of somatostatinoma." — Laboratory report interpretation