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Gastrinoma is the second most common functional pancreatic neuroendocrine tumor (pNET), with a yearly incidence of approximately 0.5 to 21.5 cases per a million of people worldwide. [5] Gastrinomas are located predominantly in the duodenum (70%) and pancreas (25%). [ 20 ]
The syndrome is caused by the formation of a gastrinoma, a neuroendocrine tumor that secretes a hormone called gastrin. [2] High levels of gastrin in the blood (hypergastrinemia) trigger the parietal cells of the stomach to release excess gastric acid. The excess gastric acid causes peptic ulcer disease and distal ulcers.
Achlorhydria induces G cell (gastrin-producing) hyperplasia, which leads to hypergastrinemia. Gastrin exerts a trophic effect on enterochromaffin-like cells (ECL cells are responsible for histamine secretion) and is hypothesized to be one mechanism to explain the malignant transformation of ECL cells into carcinoid tumors in AMAG.
Reactive gastropathy, is characterized histologically by: [2] - Foveolar hyperplasia (black arrow), as a tortuosity in the "neck" region of the gastric glands. - Scant or minimal inflammatory cells (white arrow), i.e. lack of large numbers of neutrophils and plasma cells..
A prolonged stimulation of these cells causes their hyperplasia. This is especially important in gastrinoma (the tumors in which there is an excessive secretion of gastrin), as this is one of the factors contributing to Zollinger–Ellison's syndrome. It was once believed that tumors of ECL origin form after a prolonged inhibition of gastric ...
Multiple adenomas or diffuse islet cell hyperplasia commonly occurs. About 30% of tumors are malignant and have local or distant metastases. [ 5 ] About 10-15% of islet cell tumors originate from a β-cell , secrete insulin ( insulinoma ), and can cause fasting hypoglycemia . β-cell tumors are more common in patients < 40 years of age.
*- of patients with MEN1 and gastrinoma FMTC = familial medullary thyroid cancer Micrograph of a medullary thyroid carcinoma, as may be seen in MEN 2A and MEN 2B. H&E stain. MEN 2B is sometimes known as MEN 3 and the designation varies by institution (c.f. www.ClinicalReview.com). Although a variety of additional eponyms have been proposed for ...
Histologically, the most characteristic feature is massive foveolar hyperplasia (hyperplasia of surface and glandular mucous cells). [3] The glands are elongated with a corkscrew-like appearance and cystic dilation is common. Inflammation is usually only modest, although some cases show marked intraepithelial lymphocytosis.