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Congenital adrenal hyperplasia
Primary adrenal insufficiency
Adrenal cortical carcinoma
Thyroglossal duct cyst
Thyroid eye disease (NORD)
Toxic multinodular goiter
Euthyroid sick syndrome
Subacute granulomatous thyroiditis
Growth hormone deficiency
Constitutional growth delay
Syndrome of inappropriate antidiuretic hormone secretion (SIADH)
Premature ovarian failure
Polycystic ovary syndrome
Androgen insensitivity syndrome
Autoimmune polyglandular syndrome type 1 (NORD)
Multiple endocrine neoplasia
Pancreatic neuroendocrine neoplasms
Opsoclonus myoclonus syndrome (NORD)
Adrenal insufficiency: Pathology review
Adrenal masses: Pathology review
Hyperthyroidism: Pathology review
Hypothyroidism: Pathology review
Thyroid nodules and thyroid cancer: Pathology review
Parathyroid disorders and calcium imbalance: Pathology review
Diabetes mellitus: Pathology review
Cushing syndrome and Cushing disease: Pathology review
Pituitary tumors: Pathology review
Hypopituitarism: Pathology review
Diabetes insipidus and SIADH: Pathology review
Multiple endocrine neoplasia: Pathology review
Neuroendocrine tumors of the gastrointestinal system: Pathology review
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Zollinger-Ellison syndrome p. 357
Zollinger-Ellison syndrome p. 359
Zollinger-Ellison syndrome p. 359
duodenal ulcers p. 389
gastrin in p. 380
MEN 1 syndrome p. 359
proton pump inhibitors for p. 408
Brittany Norton, MFA
Tanner Marshall, MS
Jahnavi Narayanan, MBBS
Zollinger-Ellison syndrome, named after Dr. Zollinger and Dr. Ellison - the two surgeons who first described it, is a rare endocrine disorder where there’s actually three interrelated pathologies.
First, there’s a gastrinoma, which is a gastrin-secreting tumor.
Second, the gastrinoma leads to increased gastric acid secretion from parietal cells.
Third, the excess gastric acid causes peptic ulcers.
Normally, the inner wall of the entire gastrointestinal tract is lined with mucosa, which consists of three cell layers.
The innermost layer is the epithelial layer and it absorbs and secretes mucus and digestive enzymes.
The middle layer is the lamina propria and it contains blood and lymph vessels.
The outermost layer of the mucosa is the muscularis mucosa, and it is a layer of smooth muscle that contracts and helps with the breakdown of food.
In the stomach, there are four regions - the cardia, the fundus, the body, and the pyloric antrum.
There’s also a pyloric sphincter, or valve, at the end of the stomach which closes while eating, keeping food inside for the stomach to digest.
The epithelial layer in different parts of the stomach contains different proportions of gastric glands which secrete a variety of substances.
Having said that, the cardia contains mostly foveolar cells that secrete mucus which is mostly made up of water and glycoproteins.
The fundus and the body have mostly parietal cells that secrete hydrochloric acid and chief cells that secrete pepsinogen, an enzyme that digests protein.
Finally, the antrum has mostly G cells that secrete gastrin in response to food entering the stomach.
These G cells are also found in the duodenum and the pancreas, which is an accessory organ of the gastrointestinal tract.
Gastrin stimulates the parietal cells to secrete hydrochloric acid, and also stimulates the growth of glands in the epithelial layer.
Zollinger-Ellison syndrome is a rare endocrine disorder characterized by a triad of one or more gastrinomas, increased gastric acid secretion, and peptic ulcers. The main symptom is epigastric pain from peptic ulcers, but also includes steatorrhea, weight loss, gastrointestinal bleeding, and diarrhea due to incomplete digestion and absorption. Zollinger-Ellison syndrome can be treated with acid-lowering medications and surgery to remove solitary tumors. Treatment involves reducing the production of stomach acid with medications, removing the tumor through surgery.
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