Tags

Type your tag names separated by a space and hit enter

Zollinger Ellison Syndrome

Abstract
Zollinger-Ellison syndrome (ZES) is a group of symptoms comprised of severe peptic ulcer disease, gastroesophageal reflux disease (GERD), and chronic diarrhea caused by a gastrin-secreting tumor of the duodenum or pancreas (gastrinoma triangle) that results in increased stimulation of acid-secreting cells of the stomach.[1][2] Gastrinoma is a functional neuroendocrine tumor that secretes gastric acid which causes ZES.[3] The earlier misconception was that the location of gastrinoma is in the pancreas. However, gastrinomas occur in duodenum more than pancreas by three times especially in the first portion of the duodenum.[4] There are other non-neuroendocrine tumors secreting gastrin, but not adequate amounts to cause significant symptoms.[1] Gastrinoma causing ZES occurs sporadically in about 80% of cases and is reported to be 20% to 25% as multiple endocrine neoplasia type 1 (MEN1) from multiple reports in the literature.[4][5] Approximately 50% of patients with MEN1 have ZES; therefore, MEN1 must be included in a workup if ZES is highly suspicious.[4] It takes an average of 8 years from the start of symptoms to diagnosis due to the widespread use of proton pump inhibitors (PPIs).[4][5] One study of two referral centers in Italy and the United States showed a 62% decrease in referrals and diagnosis of ZES compared to a time when PPIs were used less frequently.[5][6] In 1955, ZES made first its appearance in Annals of Surgery as a case series compiled by two surgeons from Ohio State University, Dr. Robert M. Zollinger and Edwin H. Ellison. Both cases had ulcers at the upper jejunum with excess gastric acid production refractory to medical therapy and surgical therapy including gastrectomy. The ultimate finding that led to ZES with hypersecretion, hyperacidity, and recurrent peptic ulceration was a non-beta cell islet tumor of the pancreas.[7] The pioneer case series induced enough interest to lead to the publication of multiple studies that recognize the connections between gastrinoma and ZES.

Publisher

StatPearls Publishing
Treasure Island (FL)

Language

eng

PubMed ID

30726029

Citation

Cho MS, Kasi A: Zollinger Ellison Syndrome. StatPearls. StatPearls Publishing, 2020, Treasure Island (FL).
Cho MS, Kasi A. Zollinger Ellison Syndrome. StatPearls. StatPearls Publishing; 2020.
Cho MS & Kasi A. (2020). Zollinger Ellison Syndrome. In StatPearls. Treasure Island (FL): StatPearls Publishing
Cho MS, Kasi A. Zollinger Ellison Syndrome. StatPearls. Treasure Island (FL): StatPearls Publishing; 2020.
* Article titles in AMA citation format should be in sentence-case
TY - CHAP T1 - Zollinger Ellison Syndrome BT - StatPearls A1 - Cho,Min S., AU - Kasi,Anup, Y1 - 2020/01// PY - 2019/2/7/pubmed PY - 2019/2/7/medline PY - 2019/2/7/entrez N2 - Zollinger-Ellison syndrome (ZES) is a group of symptoms comprised of severe peptic ulcer disease, gastroesophageal reflux disease (GERD), and chronic diarrhea caused by a gastrin-secreting tumor of the duodenum or pancreas (gastrinoma triangle) that results in increased stimulation of acid-secreting cells of the stomach.[1][2] Gastrinoma is a functional neuroendocrine tumor that secretes gastric acid which causes ZES.[3] The earlier misconception was that the location of gastrinoma is in the pancreas. However, gastrinomas occur in duodenum more than pancreas by three times especially in the first portion of the duodenum.[4] There are other non-neuroendocrine tumors secreting gastrin, but not adequate amounts to cause significant symptoms.[1] Gastrinoma causing ZES occurs sporadically in about 80% of cases and is reported to be 20% to 25% as multiple endocrine neoplasia type 1 (MEN1) from multiple reports in the literature.[4][5] Approximately 50% of patients with MEN1 have ZES; therefore, MEN1 must be included in a workup if ZES is highly suspicious.[4] It takes an average of 8 years from the start of symptoms to diagnosis due to the widespread use of proton pump inhibitors (PPIs).[4][5] One study of two referral centers in Italy and the United States showed a 62% decrease in referrals and diagnosis of ZES compared to a time when PPIs were used less frequently.[5][6] In 1955, ZES made first its appearance in Annals of Surgery as a case series compiled by two surgeons from Ohio State University, Dr. Robert M. Zollinger and Edwin H. Ellison. Both cases had ulcers at the upper jejunum with excess gastric acid production refractory to medical therapy and surgical therapy including gastrectomy. The ultimate finding that led to ZES with hypersecretion, hyperacidity, and recurrent peptic ulceration was a non-beta cell islet tumor of the pancreas.[7] The pioneer case series induced enough interest to lead to the publication of multiple studies that recognize the connections between gastrinoma and ZES. PB - StatPearls Publishing CY - Treasure Island (FL) UR - https://www.unboundmedicine.com/medline/citation/30726029/StatPearls:_Zollinger_Ellison_Syndrome L2 - https://www.ncbi.nlm.nih.gov/books/NBK537344 DB - PRIME DP - Unbound Medicine ER -
Try the Free App:
Prime PubMed app for iOS iPhone iPad
Prime PubMed app for Android
Prime PubMed is provided
free to individuals by:
Unbound Medicine.