PMID- 24319020 OWN - NLM STAT- MEDLINE DCOM- 20141030 LR - 20220331 IS - 1549-490X (Electronic) IS - 1083-7159 (Print) IS - 1083-7159 (Linking) VI - 19 IP - 1 DP - 2014 Jan TI - Zollinger-Ellison syndrome: classical considerations and current controversies. PG - 44-50 LID - 10.1634/theoncologist.2013-0369 [doi] AB - Zollinger-Ellison syndrome (ZES) is an endocrinopathy characterized by gastrin-secreting tumors, responsible for causing the formation of multiple, refractory, and recurrent peptic ulcers in the distal duodenum and proximal jejunum. Two main variants have been described, sporadic and those found in association with parathyroid and pituitary tumors, a genetic disorder known as multiple endocrine neoplasia-1 (MEN-1). Biochemical serum evaluation for elevated gastrin, followed by radiological or nuclear localization of the primary lesion, is mandated for establishing diagnosis. The mainstays of treatment include management of hypersecretory state with medical suppression of gastric acid production and surgical resection of primary tumor for the prevention of malignant transformation and metastatic complications. Medical therapy with proton pump inhibitors has virtually eliminated the need for acid-reducing surgical procedures. Surgical approach to sporadic and MEN-1-associated ZES varies based on our understanding of the natural history of the condition and the probability of cure; however, resection to a negative microscopic margin is indicated in both cases. Postoperative surveillance involves measurement of gastrin level, followed by imaging if elevation is detected. Re-excision of recurrent or resection of metastatic disease is a subject of controversy; however, at the present time aggressive cytoreductive approach is favored. FAU - Epelboym, Irene AU - Epelboym I AD - Department of Surgery, Columbia University Medical Center, New York, New York, USA; Department of Surgery, Hadassah-Hebrew University Medical Center, Mount Scopus, Jerusalem, Israel. FAU - Mazeh, Haggi AU - Mazeh H LA - eng PT - Journal Article PT - Review DEP - 20131206 PL - England TA - Oncologist JT - The oncologist JID - 9607837 SB - IM MH - Gastrinoma/diagnosis/therapy MH - Humans MH - Multiple Endocrine Neoplasia Type 1/diagnosis/therapy MH - Zollinger-Ellison Syndrome/*diagnosis/*therapy PMC - PMC3903066 OTO - NOTNLM OT - Gastric acid OT - Gastrinoma OT - Multiple endocrine neoplasia type 1 OT - Peptic ulcer COIS- Disclosures of potential conflicts of interest may be found at the end of this article. EDAT- 2013/12/10 06:00 MHDA- 2014/10/31 06:00 PMCR- 2015/01/01 CRDT- 2013/12/10 06:00 PHST- 2013/12/10 06:00 [entrez] PHST- 2013/12/10 06:00 [pubmed] PHST- 2014/10/31 06:00 [medline] PHST- 2015/01/01 00:00 [pmc-release] AID - theoncologist.2013-0369 [pii] AID - 13369 [pii] AID - 10.1634/theoncologist.2013-0369 [doi] PST - ppublish SO - Oncologist. 2014 Jan;19(1):44-50. doi: 10.1634/theoncologist.2013-0369. Epub 2013 Dec 6.