Surgical pathology of gastrinoma: site, size, multicentricity, association with multiple endocrine neoplasia type 1, and malignancy

Article Abstract:

Gastrinomas are tumors that secrete the peptide hormone gastrin. Most gastrinomas are malignant, but not all. Since stomach cells are stimulated by gastrin, the excess gastrin secreted by these tumors leads to the excessive secretion of both stomach acid and the powerful digestive enzyme pepsin. This lead to the development of extensive ulceration of both the stomach and the small intestines. This condition is called Zollinger-Ellison syndrome (ZES). Gastrinomas are variable in their location. They may be found at the junction of the cystic and common bile ducts, at the junction of the second and third portions of the duodenum, and where the body of the pancreas joins the neck. These regions have been called the ''gastrinoma triangle.'' Gastrinomas are not uncommon among patients with multiple endocrine neoplasia (MEN) type 1. These patients have an inherited defect that causes tumors of the pancreas, parathyroid glands, thyroid gland, and adrenal cortex. In a study of 26 patients with ZES and 18 patients with MEN type 1 and hypergastrinemia, it was found that the size and number of the gastrinomas and their distribution in the ''gastrinoma triangle'' was different for the MEN patients and the patients with sporadic disease. In six patients with MEN type 1, no gastrinomas could be found. Nine of the remaining MEN type 1 patients had gastrinomas in the duodenum; five of these nine patients had more than one duodenal gastrinoma. In contrast, the 26 patients with sporadic ZES had single gastrinomas; 14 were in the pancreas and 10 were in the duodenum. Furthermore, the patients with MEN tended to have smaller gastrinomas. These observations indicate that sporadic Zollinger-Ellison syndrome is most often associated with a single tumor in the pancreas or the duodenum. Patients with symptoms of ZES and a history indicating multiple endocrine neoplasia type 1 are more likely to have smaller multiple tumors. (Consumer Summary produced by Reliance Medical Information, Inc.)

Complications and side effects, Surgery, Genetic aspects, Adenomatosis, Familial endocrine, Multiple endocrine neoplasia, Pancreatic tumors

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Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome

Article Abstract:

The Zollinger-Ellison syndrome is a condition characterized by increased secretion of gastric (stomach) juices, severe ulcers, and gastrinomas (tumors that secrete gastrin, a digestive hormone). Many patients with this syndrome also have features characteristic of multiple endocrine neoplasia type 1 (MEN-1), a genetic disorder involving hypergastrinemia (increased gastrin levels in the blood) and gastrin-secreting tumors (gastrinomas) of the pancreas and other glands. It is possible, however, that some MEN-1 symptoms, such as hypergastrinemia, are the result of gastrinomas located in other parts of the digestive system. Biopsies of specimens from eight patients with MEN-1 and hypergastrinemia were studied microscopically to determine tumor location and size. In addition to the sites characteristic of MEN-1 (parathyroid glands, pancreas, and anterior pituitary gland), all patients had at least one gastrinoma in the duodenum, the first part of the small intestine, as well as metastatic spread to lymph nodes. In contrast to the commonly held belief about MEN-1, the tumors in the characteristic sites did not appear to secrete gastrin. The duodenal tumors were small and difficult to detect. Since the only cure for hypergastrinemia is removal of the gastrinoma, it is critical that they be accurately localized and removed. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research, Neoplastic endocrine-like syndromes

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A 58-year-old man with recurrent lower gastrointestinal bleeding for over two years

Article Abstract:

A 58-year-old man with recurrent gastrointestinal bleeding was found to have a large stromal tumor of the small intestine. In the two years prior to admission, he intermittently passed bloody stools and had evidence of iron-deficiency anemia. A colonoscopy revealed no sign of polyps, masses, inflammation or hemorrhoids. Ulcers were also ruled out. These findings as well as the patient's lack of other symptoms and the duration of the bleeding suggested he had a benign tumor of the small intestine. These are generally diagnosed in patients between 50 and 80, and about 40% of patients with benign small-intestinal tumors have gastrointestinal bleeding. The patient had exploratory surgery, and a tumor 9 centimeters (cm) by 5 cm by 4.5 cm was removed from the small intestine. Seven months following surgery, the patient was healthy and had no signs of gastrointestinal bleeding.

Tumors

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