Gastrinoma

(Zollinger-Ellison syndrome; ZE syndrome)

A gastrinoma is caused by a mostly localized in the pancreas or duodenal gastrin tumor. Hypersecretion of gastric acid and aggressive, refractory peptic ulcers are the result (Zollinger-Ellison syndrome). Diagnostically serum gastrin levels are measured. Treatment consists of proton pump inhibitors and surgical resection.

The gastrinoma is an endocrine pancreatic tumor (Endocrine pancreatic tumors) that arises from islet cells, but can also develop from gastrin cells in the duodenum and, less often, in other places in the body. 80-90% gastrinomas occur in the pancreas or in the duodenal wall. The remaining locations are the hilum, the mesentery, the stomach, lymph nodes or ovary. About 50% of patients have multiple tumors. Gastrinomas are usually small (<1 cm in diameter) and grow slowly. About 50% are malignant. Around 40-60% of patients with gastrinoma have multiple endocrine neoplasia (overview of multiple endocrine neoplasia).

A gastrinoma is caused by a mostly localized in the pancreas or duodenal gastrin tumor. Hypersecretion of gastric acid and aggressive, refractory peptic ulcers are the result (Zollinger-Ellison syndrome). Diagnostically serum gastrin levels are measured. Treatment consists of proton pump inhibitors and surgical resection. The gastrinoma is an endocrine pancreatic tumor (Endocrine pancreatic tumors) that arises from islet cells, but can also develop from gastrin cells in the duodenum and, less often, in other places in the body. 80-90% gastrinomas occur in the pancreas or in the duodenal wall. The remaining locations are the hilum, the mesentery, the stomach, lymph nodes or ovary. About 50% of patients have multiple tumors. Gastrinomas are usually small (<1 cm in diameter) and grow slowly. About 50% are malignant. Around 40-60% of patients with gastrinoma have multiple endocrine neoplasia (overview of multiple endocrine neoplasia). The symptoms and complaints Zollinger-Ellison syndrome typically falls with an aggressive peptic ulcer disease, where ulcers occur in non-standard locations (up to 25% are distal to the duodenal bulb located). However, up to 25% of patients at diagnosis have no ulcer. There may be typical Ulkussymptome and complications (eg. As perforation, bleeding, obstruction). In 25-40% of patients with diarrhea is the initial symptom. Diagnostic serum gastrin CT, scintigraphy or PET to locating the suspected gastrinoma follows a history, v. a. if the symptoms are refractory to acid secretion inhibitory standard therapy. The most reliable test is the serum gastrin levels. All patients have levels> 150 pg / ml; significantly increased levels of> 1000 pg / ml in a patient with an appropriate clinical picture and increased gastric acid secretion of> 15 mEq / h confirm the diagnosis. However, a moderate hypergastrinemia may also occur at hypochlorhydria (z. B. pernicious anemia, chronic gastritis, treatment with a proton pump inhibitor), in renal insufficiency with reduced renal gastrin secretion, after extensive intestinal resection and at a pheochromocytoma. A Sekretinprovokationstest may be useful in patients with Gastrinspiegeln <1000 pg / ml. Here is an i.v. Bolus of 2 g / kg secretin, and there are serial Gastrinspiegelbestimmungen made (10 and 1 min before and 2, 5, 10, 15, 20 and 30 min after injection). The characteristic response in a gastrinoma is an increase of gastrin, the opposite of what occurs in patients with hyperplasia of G cells in the antrum or peptic ulcer disease a. Patients should be clarified also in terms of Helicobacter pylori infection, which often leads to peptic ulcers and a moderate excess gastrin. When the diagnosis has been confirmed, a location of the tumor (or tumors) must be done. The first study is an abdominal CT or Somatostatinrezeptorszintigraphie that can identify the primary tumor and metastases. A PET or selective angiography with magnification and subtraction is also helpful. If no evidence of metastatic disease exists and the primary tumor remains unclear, an endoscopic ultrasound should be performed. Selective arterial secretin is an alternative. Prognosis If an isolated tumor was surgically removed completely, the 5- and 10-year survival rates of> 90% compared to 43% for 5 years and 25% for 10 years after incomplete resection are. Therapy inhibition of acid secretion Surgical resection for localized disease chemotherapy for metastatic disease inhibition of acid secretion proton pump inhibitors are the drugs of choice: z. As omeprazole or esomeprazole 40 mg p.o. 2 times a day. The dose may be gradually reduced in symptom-free and reduced acid secretion. A maintenance dose is required; Patients have to take these substances permanently, unless they undergo surgery. Octreotidinjektionen, 100-500 ug s.c. 2 to 3 times a day, can also inhibit the production of stomach acid and are used palliative in patients who do not respond well to proton pump inhibitors. A long-acting Octreotidpräparat may 20-30 mg i.m. werden.Operative given once a month intervention Surgical resection should be attempted in patients without evidence of metastatic disease. During surgery a duodenotomy, an endoscopic Translumination or sonography can help in tumor localization. Curative surgery is possible in 20% of patients if the gastrinoma not in the context of multiple endocrine neoplasia auftritt.Chemotherapie in patients with metastatic disease is streptozotocin in combination with 5-fluorouracil or doxorubicin, the preferred chemotherapy for islet cell tumors. It can reduce the tumor mass (in 50-60% of cases) and the serum gastrin levels and is a helpful adjunctive therapy to omeprazole. Newer chemotherapies that are studied in insulinoma include temozolomide-based regime, everolimus or sunitinib. In patients with metastatic disease, chemotherapy is not curative. Important Points Most gastrinomas manifest with symptoms of peptic ulcer disease, but some patients diarrhea occurs. About half of the patients has multiple gastrinomas, and about half has a multiple endocrine neoplasia; half of gastrinomas is malignant. Serum gastrin levels are diagnostic in general, but a Sekretinprovokationstest is indicated in patients with borderline elevated levels. Tumors can be located usually with CT, somatostatin receptor scintigraphy or PET. The acid secretion is inhibited with a proton pump inhibitor, sometimes with octreotide, unless it is carried out a surgical resection.

Health Life Media Team

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