Pathophysiology
Gastrin works on the parietal cells of the gastric glands, causing them to secrete more hydrogen ions into the stomach lumen. In addition, gastrin acts as a trophic factor for parietal cells, causing parietal cell hyperplasia. Normally, hydrogen ion secretion is controlled by a negative feedback loop by gastric cells to maintain a suitable pH, however, the neuroendocrine tumor that is present in individuals with Zollinger–Ellison Syndrome has no regulation, resulting in excessively large amounts of secretion. Thus, there is an increase in the number of acid-secreting cells, and each of these cells produces acid at a higher rate. The increase in acidity contributes to the development of peptic ulcers in the stomach, duodenum (first portion of the small bowel) and occasionally the jejunum (second portion of the small bowel), the last of which is an atypical ulcer. Diagnosis
Zollinger–Ellison syndrome may be suspected when the above symptoms prove resistant to treatment when the symptoms are especially suggestive of the syndrome, or when endoscopy is suggestive. The diagnosis is made through several laboratory tests and imaging studies:
Secretin stimulation test, which measures evoked gastrin levels. Note that the mechanism underlying this test is in contrast to the normal physiologic mechanism whereby secretin inhibits gastrin release from G cells. Gastrinoma cells release gastrin in response to secretin stimulation, thereby providing a sensitive means of differentiation.
Surgical excision may be required if its presence is resulting in atrophy of the adrenal glands and the surrounding tissues.In order to acquire better treatment strategies, it is important to further examine, study and discern the distinct molecular mechanisms involved in the formation of endogenous Adrenal Adenomas, hyperplasias, and ACTH-independent Cushings Syndrome to improve the available diagnostic and prognostic markers that can assist clinicians in the management and advance-treatment of such conditions. Prognosis
The long-term outlook for individuals diagnosed with non-functional adrenocortical adenoma is usually excellent. The long-term outlook for individuals diagnosed with functional adrenocortical adenoma is good with early diagnosis and treatment. Epidemiology
Prevalence: Female > Male
More common in adults
Relatively earlier onset in females (ages ≤ 20) than males (ages ≤ 30)
Most common cause of ACTH-independent Cushings syndrome

See also
Hyperplasia
Adrenal tumor
Cushings syndrome
Conns syndrome
Hypercortisolism
Hyperaldosteronism
Hyperandrogenism
Adrenal gland
Adrenal paraganglioma
Adrenal Pheochromocytoma
Adrenal ganglioneuroma

References


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