Zominger-Ellison Syndrome

Zominger-Ellison Syndrome

Zominger-Emison syndrome is a pancreatic islet cell tumor that produces gastrin and is accompanied by hypersecretion of hydrochloric acid and peptic ulcers. In 60% of cases, tumors are malignant, tumor sizes vary from 2 mm to 20 cm. Tumor resection is effective in about 10% of patients.

Clinical picture

• • Pain (similar to that of a peptic ulcer). Approximately 75% of ulcers are located in the duodenal bulb, in other cases – in the distal part of the duodenum or jejunum. Often find tumors of multiple localization.
  • Diarrhea develops in half of the patients due to hypersecretion of hydrochloric acid and is accompanied by significant weight loss. Increased acidity leads to damage to the mucous membrane of the small intestine, inactivates pancreatic lipase and precipitates bile acids, causing steatorrhea. A high level of gastrin leads to incomplete absorption of Na + and water, increasing intestinal motility.
  • endocrine disorders. Zominger-Emison syndrome in 20% of cases acts as a component of familial polyendocrine adenomatosis type I. Approximately 20% of patients have hyperparathyroidism, and tumors of the pituitary gland, adrenal glands, ovaries and thyroid gland are also found.

Laboratory diagnostics

• • Gastrin. Increased basal gastrin level, which does not change after an hour after eating. Intravenous secretin increases gastrin by 200 units (but does not decrease), intravenous calcium causes a significant (rather than moderate) increase in gastrin
  • Basal hydrochloric acid secretion often exceeds 10 mEq/h, and the ratio of basal to peak secretion is more than 0.6. Special Studies
  • KG and ultrasound of the abdominal organs
  • Endoscopic examination of the gastrointestinal tract
  • Determination of tumor localization is based on the selective study of gastrin levels by cannulation of pancreatic and abdominal veins. The method facilitates surgical treatment for primary multiple tumors and small tumor volumes that are not detected by conventional drugs.

Drug therapy

• • H2-histamine receptor blockers: cimetidine, starting at 300 mg every 6 hours with a gradual increase to

1.25–5.0 g/day, ranitidine starting at 150 mg 12 hours later up to 3.6 g/day, or famotidine 20 mg at bedtime, likely to increase dose to 800 mg/day (recommended doses may exceed higher daily doses of products).

• • If there is no effect, anticholinergics are additionally prescribed orally 30 minutes before meals or antacids containing aluminum and magnesium, 1 hour and 3 hours after meals and at night, or a combination of both.
  • With the development of resistance to H2-histamine receptor blockers – omeprazole 20-100 mg / day in 2 doses, then cimetidine, starting with 300 mg after 6 hours with a gradual increase to 1.25-5.0 g / day, or ranitidine starting at 150 mg 12 hours later up to 6 g/day, or famotidine 20 mg at bedtime with dose increases up to 800 mg/day allowed (recommended doses may exceed higher daily doses of products).
  • Precautions
  • In kidney disease and elderly patients, an individual selection of doses is necessary.
  • Consideration should be given to the likelihood of developing gynecomastia when taking high doses of cimetidine (> 2.4 g / day), as well as the antiandrogenic activity of H2 receptor blockers.
  • Reception of cimetidine is stopped, gradually reducing the dose.

Surgery. Total gastrectomy is the method of choice. 50% level of 10-year survival with all this is probably due to the slow progression of the lesion, tk. most deaths are associated with metastasis. Diet. Depending on the condition, diet options No. 1 are prescribed.

Complications

• • Peptic ulcers may be complicated by bleeding or perforation
  • Approximately in 2/3 cases – malignancy and metastasis.

Course and forecast

• • 5-year survival – 62-75%, 10-year – 47-53%
  • For inoperable tumors 5-year survival – 43%, 10-year – 25%
  • The prognosis is more favorable with complete resection of the tumor. Concomitant pathology
  • hyperparathyroidism
  • Prolactinoma
  • insulin ma
  • carcinoid tumors.

Synonyms

• • Adenoma of the pancreas, ulcerogenic
  • Gastrinoma

See also Adenomatosis polyendocrine ICD. E16.8 Other specified disorders of pancreatic secretion

Note

• • Hyperplasia of G-cells in the antrum of the stomach also leads to hypergastrinemia and the formation of peptic ulcers (Sominger-Emison pseudosyndrome)
  • Other sites of tumor localization are the duodenum, spleen and lymph nodes.