Zollinger-Ellison syndrome
Summary
Definition
History and exam
Key diagnostic factors
- refractory or recurrent peptic ulcer disease
- diarrhea
Other diagnostic factors
- epigastric abdominal pain
- gastroesophageal reflux disease
- steatorrhea
- gastrointestinal bleed
- bone pain
Risk factors
- multiple endocrine neoplasia type 1 (MEN1)
Diagnostic tests
1st tests to order
- fasting serum gastrin
- gastric pH
Tests to consider
- secretin stimulation test
- calcium infusion test
- glucagon test
- abdominal CT scan
- abdominal MRI scan
- somatostatin receptor scintigraphy (SRS)
- gallium(Ga)-68 DOTATATE PET
- endoscopic ultrasound (EUS)
- esophagogastroduodenoscopy (EGD)
- chest CT
- genetic testing for multiple endocrine neoplasia type 1 (MEN1)
Treatment algorithm
localized disease: sporadic
localized disease: MEN1
metastatic disease: predominantly hepatic
metastatic disease: extrahepatic
Contributors
Authors
Joseph R. Pisegna, MD
Professor of Medicine
Chief
Division of Gastroenterology, Hepatology, and Parenteral Nutrition
David Geffen School of Medicine at University of California
VA Greater Los Angeles Healthcare System
Department of Veterans Affairs
Los Angeles
CA
利益声明
JRP is an author of a number of references cited in this topic. JRP has received a grant from Takeda Pharmaceuticals and is a speaker for Phathom Pharmaceuticals and AbbVie.
鸣谢
Dr Joseph R. Pisegna would like to gratefully acknowledge Dr Deepinder Goyal, Dr Jihane Benhammou, Dr David Oh, and Dr Hank Wang, previous contributors to this topic.
利益声明
DG, JB, DO, and HW declare that they have no competing interests.
同行评议者
Peter Draganov, MD
Associate Professor of Medicine Division
Gastroenterology, Hepatology and Nutrition
University of Florida
Gainesville
FL
利益声明
PD declares that he has no competing interests.
Petros Perros, BSc, MBBS, MD, FRCP
Consultant Endocrinologist
Freeman Hospital
Newcastle-Upon-Tyne Hospitals Trust
UK
利益声明
PP declares that he has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
参考文献
关键文献
Falconi M, Eriksson B, Kaltsas G, et al. ENETS consensus guidelines update for the management of patients with functional pancreatic neuroendocrine tumors and non-functional pancreatic neuroendocrine tumors. Neuroendocrinology. 2016;103(2):153-71.全文 摘要
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].全文
Kunz PL, Reidy-Lagunes D, Anthony LB, et al. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.全文 摘要
Pavel M, Öberg K, Falconi M, et al. Gastroenteropancreatic neuroendocrine neoplasms: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jul;31(7):844-60.全文 摘要
参考文献
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
鉴别诊断
- Atrophic gastritis
- Peptic ulcer disease from Helicobacter pylori infection
- Idiopathic gastric hypersecretion
更多 鉴别诊断指南
- NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors
- Clinical practice update on management of subepithelial lesions encountered during routine endoscopy: expert review
更多 指南患者教育信息
Pancreatic cancer
Peptic ulcers
更多 患者教育信息
登录或订阅即可浏览 BMJ Best Practice 临床实践完整内容
内容使用需遵循免责声明