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Reoperative Surgery for the Zollinger-Ellison Syndrome

2010; Elsevier BV; Volume: 44; Issue: 1 Linguagem: Inglês

10.1016/j.yasu.2010.05.006

1878-0555

Stephen R. Grobmyer, Steven N. Hochwald,

Fibroblast Growth Factor Research

The Zollinger-Ellison syndrome (ZES) is a syndrome of gastric acid hypersecretion and severe peptic ulcer disease caused by a gastrin-producing neruoendocrine tumor [ [1] Zollinger R.M. Ellison E.H. Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. Ann Surg. 1955; 142 ([discussion: 724–8]): 709-723 Crossref PubMed Scopus (692) Google Scholar ]. ZES is a rare syndrome with an incidence of 1 to 3 cases per 1 × 106 people per year in the United States [ [2] Gibril F. Jensen R.T. Zollinger-Ellison syndrome revisited: diagnosis, biologic markers, associated inherited disorders, and acid hypersecretion. Curr Gastroenterol Rep. 2004; 6: 454-463 Crossref PubMed Scopus (64) Google Scholar ]. ZES occurs as either a component of multiple endocrine neoplasia type 1 (MEN-1) (20% of cases) or more commonly as sporadic disease (80% of cases) [ 3 Underdahl L.O. Woolner L.B. Black B.M. Multiple endocrine adenomas; report of 8 cases in which the parathyroids, pituitary and pancreatic islets were involved. J Clin Endocrinol Metab. 1953; 13: 20-47 Crossref PubMed Scopus (132) Google Scholar , 4 Wermer P. Genetic aspects of adenomatosis of endocrine glands. Am J Med. 1954; 16: 363-371 Abstract Full Text PDF PubMed Scopus (556) Google Scholar , 5 Thompson N.W. Bondeson A.G. Bondeson L. et al. The surgical treatment of gastrinoma in MEN I syndrome patients. Surgery. 1989; 106 ([discussion: 1085–6]): 1081-1085 PubMed Google Scholar , 6 Ellison E.C. Zollinger-Ellison syndrome: a personal perspective. Am Surg. 2008; 74: 563-571 PubMed Google Scholar ]. Approaches to treatment of patients with ZES have evolved from surgical management of the stomach [ 7 Wilson S.D. Ellison E.H. Survival in patients with the Zollinger-Ellison syndrome treated by total gastrectomy. Am J Surg. 1966; 111: 787-791 Abstract Full Text PDF PubMed Scopus (27) Google Scholar , 8 Fox P.S. Hofmann J.W. Decosse J.J. et al. The influence of total gastrectomy on survival in malignant Zollinger-Ellison tumors. Ann Surg. 1974; 180: 558-566 Crossref PubMed Scopus (63) Google Scholar , 9 Thompson J.C. Lewis B.G. Wiener I. et al. The role of surgery in the Zollinger-Ellison syndrome. Ann Surg. 1983; 197: 594-607 Crossref PubMed Scopus (133) Google Scholar ] and medical management of acid hypersecretion [ 10 McCarthy D.M. Report on the United States experience with cimetidine in Zollinger-Ellision syndrome and other hypersecretory states. Gastroenterology. 1978; 74: 453-458 PubMed Scopus (143) Google Scholar , 11 Metz D.C. Pisegna J.R. Fishbeyn V.A. et al. Control of gastric acid hypersecretion in the management of patients with Zollinger-Ellison syndrome. World J Surg. 1993; 17: 468-480 Crossref PubMed Scopus (81) Google Scholar ]. The modern approach is based on an improved understanding of the disease pathobiology of ZES and is focused on surgical removal of the site(s) of gastrinoma [ 12 Howard T.J. Zinner M.J. Stabile B.E. et al. Gastrinoma excision for cure. A prospective analysis. Ann Surg. 1990; 211: 9-14 Crossref PubMed Scopus (89) Google Scholar , 13 Norton J.A. Fraker D.L. Alexander H.R. et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999; 341: 635-644 Crossref PubMed Scopus (428) Google Scholar , 14 Norton J.A. Jensen R.T. Role of surgery in Zollinger-Ellison syndrome. J Am Coll Surg. 2007; 205: S34-S37 Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar , 15 Lorenz K. Dralle H. Surgical treatment of sporadic gastrinoma. Wien Klin Wochenschr. 2007; 119: 597-601 Crossref PubMed Scopus (10) Google Scholar ]. This approach most effectively addresses the 2 clinical problems that arise in patients with ZES: gastric acid hypersecretion and removal of tumor with metastatic potential [ [13] Norton J.A. Fraker D.L. Alexander H.R. et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999; 341: 635-644 Crossref PubMed Scopus (428) Google Scholar ]. Primary surgery in ZES is appropriately considered and performed in many patients with ZES: with curative intent; [ 13 Norton J.A. Fraker D.L. Alexander H.R. et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999; 341: 635-644 Crossref PubMed Scopus (428) Google Scholar , 16 Norton J.A. Fraker D.L. Alexander H.R. et al. Surgery increases survival in patients with gastrinoma. Ann Surg. 2006; 244: 410-419 PubMed Google Scholar ] to reduce the likelihood of developing metastatic disease; [ [13] Norton J.A. Fraker D.L. Alexander H.R. et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999; 341: 635-644 Crossref PubMed Scopus (428) Google Scholar ] to prolong survival; [ [17] Norton J.A. Jensen R.T. Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison syndrome. Ann Surg. 2004; 240: 757-773 Crossref PubMed Scopus (185) Google Scholar ] and/or to palliate symptoms associated with hypergastrinemia and its sequelae [ 17 Norton J.A. Jensen R.T. Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison syndrome. Ann Surg. 2004; 240: 757-773 Crossref PubMed Scopus (185) Google Scholar , 18 Libutti S.K. Alexander Jr., H.R. Gastrinoma: sporadic and familial disease. Surg Oncol Clin N Am. 2006; 15: 479-496 Abstract Full Text Full Text PDF PubMed Scopus (9) Google Scholar ]. Gastrinomas in sporadic disease and those arising in the setting of MEN-1 have different patterns of presentation and natural histories leading to recommendations for distinct therapeutic approaches to ZES in these 2 settings.