Secretin and Calcium Provocative Tests in the Zollinger-Ellison Syndrome

Frucht, Harold; Howard, John M.; Slaff, James I.; Wank, S.A.; McCarthy, Denis M.; Maton, Paul N.; Vinayek, Rakesh; Gardner, Jerry D.; Jensen, Robert T.

doi:10.7326/0003-4819-111-9-713

Cited by 170 publications

(98 citation statements)

References 24 publications

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“…The diagnosis required an elevated fasting serum gastrin concentration in the presence of gastric acid hypersecretion (basal acid output, Ͼ15 mEq/h in patients without previous gastric acid reducing surgery or Ͼ5 mEq/h in patients with previous gastric acid-reducing surgery) and a positive gastrin provocative test (an increase in serum gastrin Ն200 pg/ml after secretin stimulation or with calcium infusion Ն395 pg/ml) (28). Blood for serum gastrin levels measurements were obtained while the subject was fasting, and levels were determined by Bioscience Laboratories (New York, NY) and Mayo Clinical Laboratories (Rochester, MN).…”

Section: Patients and General Methodsmentioning

confidence: 99%

Prospective Study of the Natural History of Gastrinoma in Patients with MEN1: Definition of an Aggressive and a Nonaggressive Form

Gibril

Venzon

Ojeaburu

et al. 2001

The Journal of Clinical Endocrinology &Amp; Metabolism

119

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Section: Patients and General Methodsmentioning

confidence: 99%

Prospective Study of the Natural History of Gastrinoma in Patients with MEN1: Definition of an Aggressive and a Nonaggressive Form

Gibril

Venzon

Ojeaburu

et al. 2001

The Journal of Clinical Endocrinology &Amp; Metabolism

119

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“…Patients were defined as being disease-free of ZES if fasting serum gastrin levels were normal, the secretin test was negative (increase of Ͻ200 pg/mL after secretin), 40 and imaging studies, including SRS, were normal. 25 During yearly follow-up based on serial imaging studies, including SRS, it was determined whether liver metastases had developed.…”

Section: Specific Protocolmentioning

confidence: 99%

“…Patients were reevaluated 3 to 6 months after the resection with conventional imaging studies, including angiography, SRS, and functional studies to assess disease activity (fasting gastrin levels, secretin test, acid secretory tests) 12,25,36,40 and at yearly intervals thereafter. Patients were defined as being disease-free of ZES if fasting serum gastrin levels were normal, the secretin test was negative (increase of Ͻ200 pg/mL after secretin), 40 and imaging studies, including SRS, were normal.…”

Section: Specific Protocolmentioning

confidence: 99%

Comparison of Surgical Results in Patients With Advanced and Limited Disease With Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

Norton

Alexander²,

Fraker³

et al. 2001

Annals of Surgery

186

201

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ObjectiveTo determine the role of surgery in patients with Zollinger-Ellison syndrome (ZES) and multiple endocrine neoplasia type 1 (MEN1) with either limited or advanced pancreatic endocrine tumors (PETs). Summary Background DataThe role of surgery in patients with MEN1 and ZES is controversial. There have been numerous previous studies of surgery in patients with PETs; however, there are no prospective studies on the results of surgery in patients with advanced disease. MethodsEighty-one consecutive patients with MEN1 and ZES were assigned to one of four groups depending on the results of imaging studies. Group 1 (n ϭ 17) (all PETs smaller than 2.5 cm) and group 3 (n ϭ 8) (diffuse liver metastases) did not undergo surgery. All patients in group 2A (n ϭ 17; single PET 2.5-6 cm [limited disease]) and group 2B (n ϭ 31; two or more lesions, 2.5 cm in diameter or larger, or one lesion larger than 6 cm) underwent laparotomy. Tumors were preferably removed by simple enucleation, or if not feasible resection. Patients were reevaluated yearly. ResultsPancreatic endocrine tumors were found in all patients at surgery, with groups 2A and 2B having 1.7 Ϯ 0.4 and 4.8 Ϯ 1 PETs, respectively. Further, 35% of the patients in group 2A and 88% of the patients in group 2B had multiple PETs, 53% and 84% had a pancreatic PET, 53% and 68% had a duodenal gastrinoma, 65% and 71% had lymph node metastases, and 0% and 12% had liver metastases. Of the patients in groups 2A and 2B, 24% and 58% had a distal pancreatectomy, 0% and 13% had a hepatic resection, 0% and 6% had a Whipple operation, and 53% and 68% had a duodenal resection. No patient was cured at 5 years. There were no deaths. The early complication rate, 29%, was similar for groups 2A and 2B. Mean follow-up from surgery was 6.9 Ϯ 0.8 years, and during follow-up liver metastases developed in 6% of the patients in groups 2A and 2B. Groups 1, 2A, and 2B had similar 15-year survival rates (89 -100%); they were significantly better than the survival rate for group 3 (52%). ConclusionsAlmost 40% of patients with MEN1 and ZES have advanced disease without diffuse distant metastases. Despite multiple primaries and a 70% incidence of lymph node metastases, tumor can be removed with no deaths and complication rates similar to those in patients with limited disease. Further, despite previous studies showing that patients with advanced disease have decreased survival rates, in this study the patients with advanced tumor who underwent surgical resection had the same survival as patients with limited disease and patients without identifiable tumor. This suggests that surgical resection should be performed in patients with MEN1 who have ZES and advanced localized PET.

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“…3 Increase in serum gastrin with secretin is the increase in serum gastrin in pg mL Ϫ1 with two clinical units per kg given by IV push as described previously [43]. 4 Gastric acid-reducing surgery includes vagotomy with a drainage procedure or partial gastrectomy.…”

mentioning

confidence: 99%

Management of the Zollinger–Ellison syndrome in patients with multiple endocrine neoplasia type 1

Jensen¹

1998

Journal of Internal Medicine

175

169

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Zollinger-Ellison syndrome (ZES) is the most common symptomatic pancreatic endocrine tumour in patients with MEN-1. Besides the treatment of the usual endocrinopathies seen in patients with MEN-1, the treatment of the ZES requires attention be paid to controlling the gastric acid hypersecretion, to dealing with the gastrinomas per se which are malignant in 18-60% of cases, and to the diagnosis and treatment of gastric carcinoid tumours, that are increasingly seen in these patients. In this article the current management of each of the areas is reviewed and what is known or uncertain discussed, based on our studies at the NIH and data from others. Data from 231 patients including 45 with MEN-1 and 186 without MEN-1 is contrasted in this report. Gastric acid hypersecretion has been controlled in all patients medically with MEN-1 and ZES at the NIH for up to 22 years. The current drugs of choice are H ϩ -K ϩ ATPase inhibitors and twice a day dosing is recommended. Periods of parenteral drug therapy (surgery, etc.) and pregnancy require important modifications. The appropriate surgical therapy of the gastrinoma is controversial. Eighty per cent of patients have a duodenal gastrinoma and 20-30% have a pancreatic tumour. Recent studies suggest gastrinoma enucleation combined with duodenotomy rarely results in cure. Aggressive surgery (Whipple resection) can result in cure of gastrinoma but effect on survival is unclear.There are important differences in gastrinoma location, extent, and percentage with aggressive disease in patients with or without MEN-1, which are discussed. Confusion has occurred because of lack of information on the natural history of the gastrinoma compared to the other pancreatic endocrine tumours that occur in MEN-1 and survival data from patients with and without MEN-1 is contrasted. The occurrence of gastric carcinoids in patients with and without MEN-1 with ZES is contrasted and the areas of certainty and disagreement reviewed.

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Secretin and Calcium Provocative Tests in the Zollinger-Ellison Syndrome

Cited by 170 publications

References 24 publications

Prospective Study of the Natural History of Gastrinoma in Patients with MEN1: Definition of an Aggressive and a Nonaggressive Form

Prospective Study of the Natural History of Gastrinoma in Patients with MEN1: Definition of an Aggressive and a Nonaggressive Form

Comparison of Surgical Results in Patients With Advanced and Limited Disease With Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

Management of the Zollinger–Ellison syndrome in patients with multiple endocrine neoplasia type 1

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