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Zollinger Ellison Syndrome (Gastronoma)


It is the most common malignant endocrine tumor of the pancreas.

It is often located in the duodenum (most often) or pancreas.

May be associated with MEN I syndrome. Gastrinoma is the most common enteropancreatic tumor in the MEN I syndrome.

The most common symptom is abdominal pain.

Conditions suggestive of gastrinoma in patients with peptic ulcer and gastrin levels should be measured;

• Unexplained diarrhea or steatorrhea (inactivation of pancreatic enzymes due to acidity)

• Multiple ulcers or rare localizations (eg, jejunal ulcer)

• Refractory to treatment, complicated or frequent recurrence

• Post-surgical recurrence

• Association with severe esophagitis

• Thickening of gastric folds

• Endocrinological pathology, hypercalcemia or kidney stone (MEN I)

• Fasting hypergastrinemia

Diagnosis

• Fasting gastrin level is used as a screening test, it is the most sensitive test in diagnosis. A serum gastrin level > 1000 pg/ml is highly significant for diagnosis.

• The basal acid release/stimulated acid release (BAO/MAO) ratio increases (normally < 0.4 versus > 0.6 in gastrinoma).

• Secretin stimulation test

It can be used to differentiate gastrinoma from other causes of hypergastrinemia (pernicious anemia, pyloric obstruction, atrophic gastritis, PPI use, etc.).

secretin; It decreases gastrin secretion in cases of secondary hypergastrinemia, and increases it in gastrinoma.

It is the most valuable and most specific test in diagnosis.

• EUS is the most valuable method for demonstrating the tumor radiologically.

• PET-CT and somatostatin (pentreotide) scintigraphy are very useful in detecting distant metastases.

• High dose PPI is given for symptoms and ulcer healing.

• Surgery is performed in cases where the tumor can be localized.

• Somatostatin or chemotherapy is given in metastatic cases

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