Treatment: Zollinger-Ellison Syndrome

Omeprazole (or lansoprazole), beginning at 60 mg PO q a.m. and increasing until maximal gastric acid output is <10 mmol/h before next dose, is drug of choice during evaluation and in pts who are not surgical candidates; dose can often be reduced over time. Radiolabeled octreotide scanning has emerged as the most sensitive test for detecting primary tumors and metastases; may be supplemented by endoscopic ultrasonography. Exploratory laparotomy with resection of primary tumor and solitary metastases is done when possible. In pts with MEN 1, tumor is often multifocal and unresectable; treat hyperparathyroidism first (hypergastrinemia may improve). For unresectable tumors, parietal cell vagotomy may enhance control of ulcer disease by drugs. Chemotherapy is used for metastatic tumor to control symptoms (e.g., streptozocin, 5-fluorouracil, doxorubicin, or interferon α); 40% partial response rate. Newer agents effective in pancreatic neuroendocrine tumors have not been evaluated.

For a more detailed discussion, see Del Valle J: Peptic Ulcer Disease and Related Disorders, Chap. 348, p. 1911, in HPIM-19.