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Gastrinoma

Gastrinoma — gastrin - the producing tumor which is clinically shown the triad described in 1955 by Zollinger and E. Ellison: the expressed hypersecretion of hydrochloric acid covering cells of a stomach, recurrent round ulcers, not insulin - the producing pancreas tumor (the table of a gastrinom).

Etiology

Gastrinoma — in 90% of cases a malignant metastatic tumor, more often (80%) is located in PZhZh, is much more rare — in a duodenum wall (15%) and antral department of a stomach, is extremely rare (5%) — in other bodies (an epiploon, ovaries, biliary system). In 25% of cases of a gastrinom meets within a syndrome of multiple endocrine neoplaziya of 1 type.

Pathogeny

Uncontrollable products of gastrin a tumor are the cornerstone of a pathogeny of a syndrome of Zollingera-Ellison, Gipergastrinemiya leads to hyper stimulation, a hyperplasia of covering cells and significant increase in products of hydrochloric acid which cause forming of round ulcers, an inactivation of pancreatic enzymes and damage mucous intestines owing to what diarrhea develops.

Epidemiology

Gastrinoma — a rare disease, occurs no more than at 1% of patients with round ulcers. Frequency of new cases makes 0,5 on 1 million a year, more often (60%) men suffer.

Clinical manifestations

Recurrent round ulcer, refractory to usual therapy.
Diarrhea and steatorrhea (50% of cases).
Metastasises of a malignant gastrinoma in a liver and other bodies (60%).

Diagnosis

Raised at least by 3 times (norm <60 pg/ml) the level of gastrin which has to be investigated in different days.
Increase of basal level of secretion of hydrochloric acid at an intact stomach more than 15 mmol/h, and at resected more than 5 mmol/h.
Topical diagnosis of a tumor.

Differential diagnosis

Peptic ulcer of a stomach and duodenum; symptomatic ulcers.

Treatment

At a solitary high-quality gastrinoma tumor enucleation is shown. At the gastrinoma which are found sporadic the greatest possible number of radical operations makes about 30%. At nonresectable tumors or impossibility of their visualization of intervention for the purpose of a pankreatoduodenalny resection or a gastrectomy are not made to the patient only medicamentous therapy is shown. Drug of the choice of anti-proliferative therapy at malignant gastrinoma is oktreotid. For symptomatic therapy at ulcers and diarrhea blockers of a proton pomp are very effective (omeprazol).

Forecast

The five-year survival among patients with considerably resected gastrinoma makes 90%, among all patients from gastrinoma it makes 60-75%, 10-year-old — 50%. At detection of metastasises the 5-year survival decreases to 20%. The tumoral progression is a cause of death in 60% of cases.

 
"Inborn hemangioma   Gemangioperitsitoma"