Zollinger–Ellison syndrome

Zollinger- Ellison syndrome is paraneoplastic appearance, which is based on the formation of the so-called hormone gastrin by gastrinomas. This hormone stimulates the stomach to increased hydrochloric acid production. Is named the disease after the Erstbeschreibern, the American surgeon Robert Milton Zollinger (1903-1992) and Edwin Homer Ellison ( 1918-1970 ).

Cause

The Zollinger -Ellison syndrome is based on rare tumors in the pancreas (75%) or in the upper part of the small intestine (20%) and malignant in 60 % of cases. These tumors produce the hormone gastrin and are called gastrinomas also. Gastrinomas are among the neuroendocrine tumors ( NET), they can occur singly or as multiple tumors. About half to two-thirds of the single gastrinomas are malignant (malignant ) tumors that can absiedeln in liver and lymph nodes. Almost 25 % of patients with gastrinomas have as part of multiple endocrine neoplasia ( MEN) type I multiple tumors: the three Ps pituitary (English for pituitary ), pancreas, parathyroid ( parathyroid ).

Produced by a gastrinoma gastrin causes an overproduction of stomach acid. Gastrin acts on the stomach to the Parietal-/Belegzellen, causing them to release more hydrogen ions into the gastric lumen ( proton pump). The increase of acidity contributes to the development of gastric ulcers in ( gastric ulcers ) and duodenal ulcer ( duodenal ulcers ), since Helicobacter paradoxically thrives in acidic better.

Symptoms

• Abdominal pain: ulcers of the stomach or duodenum, and partly also in the jejunum (atypical localization )
• Gastroesophageal Reflux
• Occasional vomiting of blood ( hematemesis )
• Diarrhea in about half the cases,
• Occasionally fatty stools, as hydrochloric acid inactivated the lipases
• Hypokalemic metabolic alkalosis in the individual case
• Very rare hyperparathyroidism ( MEN- independent)

Diagnosis

For the diagnosis, especially the increased gastrin ( hypergastrinemia ) is leading the way. Values ​​above 1000 ng / l are almost conclusive. In contrast to other diseases in which the gastrin can also be increased, it is Zollinger -Ellison syndrome, in addition after challenge with a secretin often 100 percent increase in gastrin. Neuroendocrine tumors typically form chromogranin, which is detected in the serum.

Furthermore, the drug-resistant, often atypical localized ulcers can be used to confirm the diagnosis.

The diagnostic localization occurs about with octreotide scintigraphy, positron emission tomography, MRI, CT and endoluminal ultrasound.

Therapy

Proton pump inhibitors reduce acid secretion. If possible, the tumors to be removed surgically. Curative However, this is possible only in the absence of metastases. Chemotherapy has not proven to be effective. The hormone production can be inhibited by somatostatin.

Metastatic neuroendocrine tumors can be treated with a palliative approach with 90Y - DOTATOC or 177Lu - DOTATOC.

Occurrence

This disease is extremely rare and only occurs in about five to ten cases per year per one million people. It can occur at any age, but mainly at 30 - to 60 -year-olds.