Related articles: Zollinger-Ellison syndrome
Definition
Zollinger-Ellison syndrome is a serious pathological condition characterized by increased secretion of gastric acids, peptic ulcerations and marked hypergastrinemia. These phenomena are secondary to the presence of a gastrin-secreting tumor (called gastrinoma) located in the duodenal wall, in the pancreas, in the abdominal lymph nodes or, rarely, in ectopic sites (heart, ovary and liver).
In about 75% of cases, Zollinger-Ellison syndrome is sporadic, but may also be associated with multiple endocrine neoplasia 1 (MEN1), which depends on specific gene mutations transmitted with an autosomal dominant pattern.
Usually, Zollinger-Ellison syndrome is diagnosed between 30 and 50 years of age.
The typical aspect that is found in Zollinger-Ellison syndrome is the presence of numerous recurrent and / or resistant gastric and duodenal ulcers to the conventional treatment.
Most common symptoms and signs *
- Anorexia
- Retrosternal burning
- Heartburn
- Bad digestion
- Diarrhea
- Yellow Diarrhea
- Abdominal pain
- Pain in the upper part of the abdomen
- Hematemesis
- Gastrointestinal hemorrhage
- Hypokalemia
- Mucorrea
- Nausea
- Weight loss
- Gastrointestinal perforation
- Acid regurgitation
- steatorrhea
- He retched
Further indications
The most common symptoms are pain in the upper abdomen, diarrhea and gastro-esophageal reflux. Gastric hypersecretion often causes persistent heartburn and can complicate peptic ulcer with hemorrhage and gastrointestinal perforation.
Other symptoms include nausea, vomiting (or hematemesis), intestinal malabsorption and weight loss.
Initially, the diagnosis of Zollinger-Ellison syndrome is based on the clinical picture. Confirmation is obtained by measuring fasting gastrinemia levels (FSG), which are almost always high, and by detecting a gastric pH below 2. To localize the gastrinoma, imaging tests are also indicated (abdominal ultrasound). or endoscopic, computerized axial tomography and somatostatin receptor scintigraphy).
The treatment involves the short and long term control of gastric acid hypersecretion with proton pump inhibitors and histamine H2 receptor antagonists.
Where possible, localized gastrinoma should be surgically removed. In the case of metastatic cancer, on the other hand, various approaches can be attempted, such as chemotherapy, embolization of hepatic neoplastic masses and more aggressive surgical procedures.
In the absence of liver metastases, the prognosis is good, while the survival rate is reduced in the presence of multiple tumors or poorly responding to medical therapy.
