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Gastrinoma belongs to the neuroendocrine tumors. Most often it is located in the duodenum or the head of the pancreas. It causes a group of characteristic symptoms known as Zollinger-Ellison Syndrome. What are neuroendocrine tumors? How does gastrinoma cause symptoms? Is there a treatment?

Gastrinomais one of the rare tumors. The incidence is 1: 1,000,000 / year. It is slightly more common in men, and the average age at which symptoms appear is 43 years. Usually it is a small tumor not exceeding 1 cm. More than half are multifocal. In ⅔ of cases it is malignant and may metastasize early, most often to the lymph nodes, liver, spleen, mediastinum and bones. Typical locations are the duodenum or pancreas (80%), other rarer locations include the stomach, liver, and even ovaries.

Gastrinoma: causes

In approximately 65 percent of cases, gastrinomas appear as sporadic tumors. In other cases, they are one of the components of the MEN1 syndrome. MEN1 or multiple neoplasms of the endocrine system type 1. It is an inherited disease caused by a mutation in the MEN 1 gene. Apart from a neuroendocrine tumor of the pancreas, it is characterized by the coexistence of tumors of the anterior pituitary gland and primary hyperparathyroidism.

Gastrinoma: symptoms

The result of the production of gastrin by the tumor is overstimulation of parietal cells to produce hydrochloric acid. Not all patients have enough hormone production to cause symptoms, which can significantly delay diagnosis of the disease.

About 50 percent of patients develop Zollinger-Ellison syndrome due to excess gastrin. The primary symptom is recurrent peptic ulcers, which are often at an unusual location and do not respond to standard therapy. There is diarrhea, often fatty diarrhea. Other features suggesting Zollinger-Ellison syndrome include severe oesophagitis, coexistence of pancreatic isletoma, pituitary tumor, or hyperparathyroidism. Since the tumor produces ACTH in some cases, we can observe the symptoms of Cushing's syndrome.

Gastrinoma: diagnosis

  • Laboratory tests

a. high levels of gastrinb. if the result raises doubts, the so-called secretin test or gastrinic stimulation test. ratingbasal gastric secretion (BAO) 15 mmol / ld. there may be abnormalities typical of other neoplasms included in the MEN1 syndrome (e.g. hypercalcemia)

  • Endoscopy

hypertrophy of folds of the gastric mucosa (>90%), ulcers in the upper gastrointestinal tract (~ 75% in the duodenal bulb)

  • Imaging tests

- receptor scintigraphy and endosonography (sensitivity 80%) - ultrasound - CT (computed tomography) - MRI (magnetic resonance) - selective arteriography (sensitivity in sporadic tumors ~ 50%)

Gastrinoma: differential diagnosis

Consider conditions with increased gastrin concentration:

  • pernicious anemia and G-cell growth in the pre-antral part of the stomach (e.g. in atrophic gastritis, H. pylori infection and in conditions after gastrectomy with preservation of the pre-antral part).

Gastrinoma: treatment

Treatment has two main goals: 1. Symptomatic treatment of gastrointestinal ulceration based on the use of proton pump inhibitors in increased doses: eg omeprazole 60-120 mg / day, lansoprazole 75 mg / day. 2. Causal treatment consisting in resection of the tumor. If the tumor has not been located with the help of imaging tests, laparotomy is performed with careful inspection of the abdominal cavity. In advanced cases, palliative chemotherapy is used.

Gastrinoma: prognosis

After tumor removal, approximately 50 percent of patients remain in good he alth for many years. 5-year survival is achieved by 90 percent of patients, and in 60 percent of cases it is 15 years.

Worth knowing

Neuroendocrine tumors (NET)

are atypical neoplasms that originate in endocrine cells (cells of the endocrine system) found throughout the human body. They are characterized by the ability to produce biogenic hormones or amines. True to its name, a gastrinoma secretes gastrin, but it can also produce ACTH.

What is gastrin and ACTH?

Gastryna is a hormone that affects the proper functioning of the digestive system. Physiologically produced by G cells located in the pyloric part of the stomach and in the initial part of the duodenum. Influences the production of hydrochloric acid and the proper condition of the gastric mucosa.

ACTH or adrenocorticotropic hormone (corticotropin) properly secreted by the pituitary gland, which, by influencing the adrenal cortex, increases the secretion of cortisol and weakly-acting androgens.

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Category:

Oncology