Gastrinoma

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Gastrinoma
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Specialty scores for Gastrinoma

Overview

Gastrinoma is rare neuroendocrine tumor that forms in the pancreas or duodenum. These growths begin in the cells that produce gastrin, which is a hormone responsible for secretion of gastric acid. In the presence of a gastrinoma, the body secretes large amounts of gastrin hormone, resulting in excessive level of stomach acid. This can cause the ulcer formation in the stomach and small bowel. Gastrinomas can be either benign or malignant. More than 60% of gastrinomas are cancerous in nature, according to the Center for Pancreatic and Biliary Diseases.

Causes:

Gastrinomas are caused due to uncontrolled multiplication of cells that produce gastrin. The exact cause of gastrinoma is still unknown, although it is postulated that there may be an underlying genetic component, possibly in association with environmental factors. Approximately 25-30% of gastrinomas are associated with multiple endocrine neoplasia type 1 (MEN1), an inherited genetic disorder.

Signs and symptoms:

Primarily, signs and symptoms of gastrinomas are due to increase in stomach acid production. Some people experience the symptoms for several years before the definitive diagnosis is made. Symptoms and signs of a gastrinoma include abdominal pain, vomiting, heartburn, nausea, weight loss, loss of appetite, bloating, indigestion or heartburn and diarrhea. In the presence of excessive stomach acid, ulcers can form. The chances of gastrinoma are high in case of persistent ulcer, hypercalcemia (high calcium level), a family history of gastrinoma, intestinal perforation and bleeding and excessive stomach acid that doesn’t improve with treatment.

Complications:

In case the treatment is insufficient, there may be formation of additional ulcer, reduced pancreatic function and intestinal perforation.

Diagnosis:

Gastrinoma is diagnosed by costellation of symptoms and signs and several laboratory tests and imaging studies. Secretin stimulation test measures evoked gastrin levels. In response to secretin, gastrinoma cells release gastrin, thereby providing a sensitive means of differentiation. Fasting gastrin levels must be done on at least three separate occasions and should be high. Gastric acid secretion and pH provide excellent diagnostic clue (in gastrinoma patients, it is usually more than 15 mEq/hour). An increased level of chromogranin A is another common marker of neuroendocrine tumors. In addition, the size and extent of tumor must be determined using CT scan or MRI or somatostatin receptor scintigraphy. Gastrinoma tumors can be small, so imaging tests may not produce an image in every case. In such cases, an endoscopic ultrasound is performed to probe the presence of tumors in the stomach or small intestines. If a tumor is discovered, the next step is a tissue biopsy, which provides definitive diagnosis.

Management:

Treatment of gastrinoma depends on the location of tumors, the status of their spread to the nearby and distant organs and overall health of the patient. Surgery is the mainstay of treatment, and the objective of surgery is to remove the cancerous tumor from the body and cure the disease. Surgical options may include removal of the entire tumor, removal of the head of the pancreas or the tail of the pancreas, small bowel resection and removal of tumors that have spread to other organs, such as the liver or lymph nodes. In case the cancer spreads and becomes incurable or if secondary liver cancer is developed from gastrinoma, treatment options include radiofrequency ablation, transarterial chemoembolization and selective internal radiotherapy. Other treatments for gastrinomas include chemotherapy and proton-pump inhibitors that reduce the stomach acid production.

Other names of the condition:

Zollinger-Ellison syndrome

Pancreatic Ulcerogenic Tumor Syndrome

ZES

Z-E Syndrome

FDA Approved Medicines

  • FDA approved secretin, sold as SecreFlo, manufactured by Repligen as to aid in the diagnosis of pancreatic dysfunction and gastrinoma ( Gastrinoma | Pancreatic Cancer ) in year 2002.