Serum Gastrin Levels in Malignant Zollinger-Ellison Syndrome after Total Gastrectomy and Hypophysectomy

Friesen, Stanley R.; Bolinger, Robert E.; Pearse, A. G. E.; McGuigan, James E.

doi:10.1097/00000658-197009000-00014

Cited by 61 publications

(9 citation statements)

References 24 publications

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“…Literature BAO data are from 129 patients 11,14,35,[39][40][41]67,92,95,96,100,103,117,122,144,146,153,154,170,175,177,185,189,191,192,216,220,228,230,236,254,257,270,284,289,292,331,343,355,[369][370][371][372]378,401,439,443,446,474,491 ; and literature MAO data are from 55 patients 11,…”

Section: Discussionmentioning

confidence: 99%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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In patients with multiple endocrine neoplasia type 1 (MEN1), the most common functional pancreatic endocrine tumor (PET) syndrome is Zollinger-Ellison syndrome (ZES). ZES has been well studied in its sporadic form (that is, without MEN1); however, there are limited data on patients with MEN1 and ZES (MEN1/ZES), and the long-term natural history is largely unknown. To address this issue we report the results of a prospective long-term National Institutes of Health (NIH) study of 107 MEN1/ZES patients and compare our results with those of 1009 MEN1/ZES patients in 278 case reports and small series in the literature. Patients were clinically, radiologically, and biochemically evaluated yearly for all MEN1 manifestations (mean follow-up, 10 yr; range, 0.1-31 yr). Compared with patients from the literature, the NIH MEN1/ZES patients more frequently had pituitary (60%) and adrenal (45%) disease and carcinoid tumors (30%), but had equal frequency of hyperparathyroidism (94%), thyroid disease (6%), or lipomas (5%). Twenty-five percent of both the NIH and the literature patients lacked a family history of MEN1; ZES was the initial clinical manifestation of MEN1 in 40%. ZES onset preceded the diagnosis of hyperparathyroidism in 45%. However, ZES was rarely (8%) the only initial manifestation of MEN1 if careful testing was done. ZES occurred before age 40 years in 50%-60% of the current patients, in contrast to older studies. The diagnosis of ZES is delayed 3-5 years from its onset and is delayed as long as in sporadic ZES cases. Pituitary disease and carcinoid tumors (gastric > bronchial, thymic) are more frequent than generally reported, whereas a second functional PET is uncommon. In patients with MEN1/ZES without a family history of MEN1, the MEN1 manifestations are not as severe. This study shows that MEN1/ZES patients differ in many aspects from those commonly reported in older studies involving few MEN1/ZES patients. In this study we have identified a number of important clinical and laboratory features of MEN1/ZES that were not previously appreciated, which should contribute to earlier diagnosis and improve both short- and long-term management.

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Section: Discussionmentioning

confidence: 99%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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“…Increased gastric acid output has been observed in acromegaly, and immunofluorescence and ultrastructural studies revealing the underlying antral G cell hyperplasia have been made by Pearse and Bussolati (1970) and by Creutzfeldt et al (1971). A pituitary influence on islet cells, and on endocrine polypeptide neoplasms, has been postulated by Friesen et al (1970) and by Friesen (1972) following the observation that after hypophysectomy in Z-E syndrome with tumour a decrease in the size of metastases was observed, together with a fall in serum gastrin levels.…”

Section: Discussionmentioning

confidence: 99%

Two types of Zollinger-Ellison syndrome: immunofluorescent, cytochemical and ultrastructural studies of the antral and pancreatic gastrin cells in different clinical states

Polak¹,

Stagg²,

Pearse³

1972

Gut

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SUMMARY In this survey the antral, pancreatic and, where present, the neoplastic gastrin cells, were studied in eight cases of the Zollinger-Ellison syndrome. The antral G cells alone were studied in one case of Z-E syndrome, seven cases of simple duodenal ulcer, and five cases of pernicious anaemia.The Z-E cases were divided into two numerically equal groups. The first group had 'short' histories, high serum gastrin levels, and profound antral G cell hyperplasia. The second group had 'long' histories, relatively lower serum gastrin levels, normal antral G cells, and either pancreatic D cell hyperplasia or gastrinoma.Antral G cell hyperplasia, with maximal gastrin storage and normal serum gastrin levels, was found in the duodenal ulcer cases. Antral G cell hyperplasia with minimal storage and high serum gastrin levels was observed in the cases of pernicious anaemia.On the basis of our findings we propose that there exist at least two distinct types (or perhaps stages) of the Z-E syndrome. Suggestions for their pathogenesis are offered.The combination of gastric hypersecretion, intractable peptic ulceration, and non-insulin-producing pancreatic islet cell tumours was noted by Sailer and Zinniger (1946), Forty and Barrett (1952), and by Strom (1953

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“…Gastrinomas commonly arise from within the pancreas; about 5.6% of patients have a primary gastrinoma located in an ectopic site (5), including the body of the stomach, jejunum, peripancreatic lymph nodes, splenic hilum, root of the mesentery, omentum, liver, gallbladder, common bile duct, and the ovary (4,(6)(7)(8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%