Zollinger-Ellison syndrome
| Zollinger–Ellison syndrome | |
|---|---|
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| Endoscopy image of multiple small ulcers in the distal duodenum in a patient with Zollinger–Ellison syndrome | |
| Classification and external resources | |
| Specialty | endocrinology |
| ICD-10 | E16.4 |
| ICD-9-CM | 251.5 |
| MedlinePlus | 000325 |
| eMedicine | med/2437 ped/2472 |
| Patient UK | Zollinger–Ellison syndrome |
| MeSH | D015043 |
Zollinger–Ellison syndrome (ZES) is a " Gastrinoma" which is a neuroendocrine tumor that secretes Gastrin; resultant hypergastrinemia causes gastric acid hypersecretion. This leads to growth of gastric mucosa, increase in number of Parietal cells and proliferation of gastric ECL cells. Most Gastrinomas are present in duodenum (50-70%), pancreas ( 20-40%) and other intraabdominal site. ZES may occur sporadically or as part of an autosomal dominant familial syndrome called multiple endocrine neoplasia type 1 (MEN 1). The primary tumor is usually located in the pancreas, duodenum or abdominal lymph nodes, but ectopic locations have also been described (e.g., heart, ovary, gallbladder, liver, kidney).
Patients with Zollinger–Ellison syndrome may experience abdominal pain and diarrhea. The diagnosis is also suspected in patients without symptoms who have severe ulceration of the stomach and small bowel, especially if they fail to respond to treatment.
Gastrinomas may occur as single tumors or as multiple, small tumors. About one-half to two-thirds of single gastrinomas are malignant tumors that most commonly spread to the liver and lymph nodes near the pancreas and small bowel. Nearly 25 percent of patients with gastrinomas have multiple tumors as part of a condition called multiple endocrine neoplasia type I (MEN I). MEN I patients have tumors in their pituitary gland and parathyroid glands, in addition to tumors of the pancreas.
Gastrin works on the parietal cells of gastric glands causing them to secrete more hydrogen ions into the stomach lumen. In addition, gastrin acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Thus there is an increase in the number of acid-secreting cells, and each of these cells produces acid at a higher rate. The increase in acidity contributes to the development of multiple peptic ulcers in the stomach and duodenum (first portion of the small bowel).
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