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Surgery to cure the Zollinger-Ellison syndrome.
N Engl J Med. 1999 Aug 26; 341(9):635-44.NEJM

Abstract

BACKGROUND AND METHODS

The role of surgery in patients with the Zollinger-Ellison syndrome is controversial. To determine the efficacy of surgery in patients with this syndrome, we followed 151 consecutive patients who underwent laparotomy between 1981 and 1998. Of these patients, 123 had sporadic gastrinomas and 28 had multiple endocrine neoplasia type 1 with an imaged tumor of at least 3 cm in diameter. Tumor-localization studies and functional localization studies were performed routinely. All patients underwent surgery according to a similar operative protocol, and all patients who had surgery after 1986 underwent duodenotomy.

RESULTS

The 151 patients underwent 180 exploratory operations. The mean (+/-SD) follow-up after the first operation was 8+/-4 years. Gastrinomas were found in 141 of the patients (93 percent), including all of the last 81 patients to undergo surgery. The tumors were located in the duodenum in 74 patients (49 percent) and in the pancreas in 36 patients (24 percent); however, primary tumors were found in lymph nodes in 17 patients (11 percent) and in another location in 13 patients (9 percent). The primary location was unknown in 24 patients (16 percent). Among the patients with sporadic gastrinomas, 34 percent were free of disease at 10 years, as compared with none of the patients with multiple endocrine neoplasia type 1. The overall 10-year survival rate was 94 percent.

CONCLUSIONS

All patients with the Zollinger-Ellison syndrome who do not have multiple endocrine neoplasia type 1 or metastatic disease should be offered surgical exploration for possible cure.

Links

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Authors+Show Affiliations

Norton JA
Department of Surgery, University of California, San Francisco, and the San Francisco Veterans Affairs Medical Center, USA.
Fraker DL
No affiliation info available
Alexander HR
No affiliation info available
Venzon DJ
No affiliation info available
Doppman JL
No affiliation info available
Serrano J
No affiliation info available
Goebel SU
No affiliation info available
Peghini PL
No affiliation info available
Roy PK
No affiliation info available
Gibril F
No affiliation info available
Jensen RT
No affiliation info available

MeSH

AdultDisease-Free SurvivalDuodenal NeoplasmsFemaleFollow-Up StudiesGastrinomaHumansLymphatic DiseasesMaleMiddle AgedMultiple Endocrine Neoplasia Type 1Pancreatic NeoplasmsSurvival RateZollinger-Ellison Syndrome

Pub Type(s)

Clinical Trial
Journal Article

Language

eng

PubMed ID

10460814

Citation

Norton, J A., et al. "Surgery to Cure the Zollinger-Ellison Syndrome." The New England Journal of Medicine, vol. 341, no. 9, 1999, pp. 635-44.
Norton JA, Fraker DL, Alexander HR, et al. Surgery to cure the Zollinger-Ellison syndrome. N Engl J Med. 1999;341(9):635-44.
Norton, J. A., Fraker, D. L., Alexander, H. R., Venzon, D. J., Doppman, J. L., Serrano, J., Goebel, S. U., Peghini, P. L., Roy, P. K., Gibril, F., & Jensen, R. T. (1999). Surgery to cure the Zollinger-Ellison syndrome. The New England Journal of Medicine, 341(9), 635-44.
Norton JA, et al. Surgery to Cure the Zollinger-Ellison Syndrome. N Engl J Med. 1999 Aug 26;341(9):635-44. PubMed PMID: 10460814.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Surgery to cure the Zollinger-Ellison syndrome. AU - Norton,J A, AU - Fraker,D L, AU - Alexander,H R, AU - Venzon,D J, AU - Doppman,J L, AU - Serrano,J, AU - Goebel,S U, AU - Peghini,P L, AU - Roy,P K, AU - Gibril,F, AU - Jensen,R T, PY - 1999/8/26/pubmed PY - 2000/5/20/medline PY - 1999/8/26/entrez SP - 635 EP - 44 JF - The New England journal of medicine JO - N Engl J Med VL - 341 IS - 9 N2 - BACKGROUND AND METHODS: The role of surgery in patients with the Zollinger-Ellison syndrome is controversial. To determine the efficacy of surgery in patients with this syndrome, we followed 151 consecutive patients who underwent laparotomy between 1981 and 1998. Of these patients, 123 had sporadic gastrinomas and 28 had multiple endocrine neoplasia type 1 with an imaged tumor of at least 3 cm in diameter. Tumor-localization studies and functional localization studies were performed routinely. All patients underwent surgery according to a similar operative protocol, and all patients who had surgery after 1986 underwent duodenotomy. RESULTS: The 151 patients underwent 180 exploratory operations. The mean (+/-SD) follow-up after the first operation was 8+/-4 years. Gastrinomas were found in 141 of the patients (93 percent), including all of the last 81 patients to undergo surgery. The tumors were located in the duodenum in 74 patients (49 percent) and in the pancreas in 36 patients (24 percent); however, primary tumors were found in lymph nodes in 17 patients (11 percent) and in another location in 13 patients (9 percent). The primary location was unknown in 24 patients (16 percent). Among the patients with sporadic gastrinomas, 34 percent were free of disease at 10 years, as compared with none of the patients with multiple endocrine neoplasia type 1. The overall 10-year survival rate was 94 percent. CONCLUSIONS: All patients with the Zollinger-Ellison syndrome who do not have multiple endocrine neoplasia type 1 or metastatic disease should be offered surgical exploration for possible cure. SN - 0028-4793 UR - https://www.unboundmedicine.com/medline/citation/10460814/Surgery_to_cure_the_Zollinger_Ellison_syndrome_ L2 - https://www.nejm.org/doi/10.1056/NEJM199908263410902?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -