ZOLLINGER-ELLISON (Z-E) SYNDROME (GASTRINOMA)

Consider when ulcer disease is severe, refractory to therapy, associated with ulcers in atypical locations, or associated with diarrhea. Tumors are usually pancreatic or in duodenum (submucosal, often small), may be multiple, slowly growing; >60% malignant; 25% associated with MEN 1, i.e., multiple endocrine neoplasia type 1 (gastrinoma, hyperparathyroidism, pituitary neoplasm), often duodenal, small, multicentric, less likely to metastasize to liver than pancreatic gastrinomas but often metastasize to local lymph nodes.

DIAGNOSIS

Suggestive

Basal acid output >15 mmol/h; basal/maximal acid output >60%; large mucosal folds on endoscopy or upper GI radiograph.

Confirmatory

Serum gastrin >1000 ng/L or rise in gastrin of 200 ng/L following IV secretin and, if necessary, rise of 400 ng/L following IV calcium (Table 147-4).

TABLE 147-4: DIFFERENTIAL DIAGNOSTIC TESTS
  Gastrin Response to
ConditionFasting GastrinIV SecretinFood
DUN (≤150 ng/L)NCSlight ↑
Z-E↑↑↑↑↑↑NC
Antral G (gastrin) cell hyperplasia↑, NC↑↑↑
Abbreviations: DU, duodenal ulcer; N, normal; NC, no change; Z-E, Zollinger-Ellison syndrome.

DIFFERENTIAL DIAGNOSIS

Increased Gastric Acid Secretion

Z-E syndrome, antral G-cell hyperplasia or hyperfunction (? due to H. pylori), postgastrectomy retained antrum, renal failure, massive small bowel resection, chronic gastric outlet obstruction.

Normal or Decreased Gastric Acid Secretion

Pernicious anemia, chronic gastritis, gastric cancer, vagotomy, pheochromocytoma.

Treatment

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.

Outline

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Citation

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TY - ELEC T1 - ZOLLINGER-ELLISON (Z-E) SYNDROME (GASTRINOMA) ID - 623366 Y1 - 2017 PB - Harrison's Manual of Medicine UR - https://harrisons.unboundmedicine.com/harrisons/view/Harrisons-Manual-of-Medicine/623366/all/ZOLLINGER_ELLISON__Z_E__SYNDROME__GASTRINOMA_ ER -