Zollinger-ellison syndrome with interesting variations

Guida, Peter; Todd, Jean Elizabeth; Moore, Shirley; Beal, John M.

doi:10.1016/0002-9610(66)90130-9

Cited by 27 publications

(12 citation statements)

References 28 publications

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“…In contrast to the past, gastric acid hypersecretion 138,151,175,207,209,336,502,504 now can be treated effectively medically in almost every patient with ZES 199,203,204,207,277,280,488 ; no patient died of its effects in the current study. Similar conclusions to control of gastric acid secretion exist for the control of the pituitary disease, or the control of the hyperparathyroidism and prevention of the resultant renal failure, which were important contributing factors in the deaths of these patients in the past 58,62,125,133,253,468,484 .…”

Section: Discussioncontrasting

confidence: 70%

“…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%

“…Five of the 107 NIH patients with MEN1/ZES did not have hyperparathyroidism. The 240 patients in the literature were from 146 reports 1,12,16,22,31,40,47,50,63,65,70,[72][73][74]79,87,93,94,96,[101][102][103][104][107][108][109]116,120,121,123,126,130,132,135,148,149,152,157,161,170,[173][174][175]177,179,183,186,192,195,210,211,…”

Section: Methodsmentioning

confidence: 99%

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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: Discussioncontrasting

confidence: 70%

Section: Discussionmentioning

confidence: 99%

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Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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“…The search for these patients included primarily reports since 1980 when widespread availability of effective medical/surgical treatments for the gastric acid hypersecretion of MEN1 patients with ZES became available and was generally used. Specifically, patients in series or any report where the long-term survival was limited due to death from the complications of uncontrolled acid-peptic disease were excluded, because especially in many early series including patients treated prior to 1980 43,60,158,225,260,265,275,307,332,366,399,404,438,445,458 , this was the major cause of death and thus few patients had long term follow-up, which is not the case at present. Since the mid 1970’s adequate medical antisecretory treatments with either H 2 -histamine receptor antagonists or proton pump inhibitors (PPIs) became generally available 15,69,73,108,135,192,224,254,278,282,287,444,483 and this in addition to the use of total gastrectomy in selected patients, has led to the result that at present few patients die of acid-peptic related disease and the current natural history and causes of death are though to differ markedly from these early reports 54,57,78,88,150,217,367,378,379,448,465 .…”

Section: Methodsmentioning

confidence: 99%

“…These changes in treatment are widespread and have affected almost all aspects of treatment and therefore are likely having a prominent effect on changing the late course of disease in many patients and the causes of death. These changes include the increased ability to control various hormone-excess states that were a frequent cause of death in early studies of MEN1 patients 15,43,72,73,83,88,158,180,188,224,225,240,254,279,307,353,366,386,399,438,444,458,461,465,466 , accounting for up to 73–91% of all deaths in some early series 15,195,224,254,444 . These include particularly the ability to treat the gastric acid hypersecretion in Zollinger-Ellison syndrome, earlier diagnosis/better treatment of insulinomas, and the increased use of somatostatin analogues or other treatments of other hypersecretory states (I.e.…”

Section: Introductionmentioning

confidence: 99%

Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

et al. 2013

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Multiple endocrine neoplasia type 1 (MEN1) is classically characterized by the development of functional or nonfunctional hyperplasia or tumors in endocrine tissues (parathyroid, pancreas, pituitary, adrenal). Because effective treatments have been developed for the hormone excess state, which was a major cause of death in these patients in the past, coupled with the recognition that nonendocrine tumors increasingly develop late in the disease course, the natural history of the disease has changed. An understanding of the current causes of death is important to tailor treatment for these patients and to help identify prognostic factors; however, it is generally lacking.To add to our understanding, we conducted a detailed analysis of the causes of death and prognostic factors from a prospective long-term National Institutes of Health (NIH) study of 106 MEN1 patients with pancreatic endocrine tumors with Zollinger-Ellison syndrome (MEN1/ZES patients) and compared our results to those from the pooled literature data of 227 patients with MEN1 with pancreatic endocrine tumors (MEN1/PET patients) reported in case reports or small series, and to 1386 patients reported in large MEN1 literature series. In the NIH series over a mean follow-up of 24.5 years, 24 (23%) patients died (14 MEN1-related and 10 non-MEN1-related deaths). Comparing the causes of death with the results from the 227 patients in the pooled literature series, we found that no patients died of acute complications due to acid hypersecretion, and 8%–14% died of other hormone excess causes, which is similar to the results in 10 large MEN1 literature series published since 1995. In the 2 series (the NIH and pooled literature series), two-thirds of patients died from an MEN1-related cause and one-third from a non-MEN1-related cause, which agrees with the mean values reported in 10 large MEN1 series in the literature, although in the literature the causes of death varied widely. In the NIH and pooled literature series, the main causes of MEN1-related deaths were due to the malignant nature of the PETs, followed by the malignant nature of thymic carcinoid tumors. These results differ from the results of a number of the literature series, especially those reported before the 1990s. The causes of non-MEN1-related death for the 2 series, in decreasing frequency, were cardiovascular disease, other nonendocrine tumors > lung diseases, cerebrovascular diseases. The most frequent non-MEN1-related tumor deaths were colorectal, renal > lung > breast, oropharyngeal. Although both overall and disease-related survival are better than in the past (30-yr survival of NIH series: 82% overall, 88% disease-related), the mean age at death was 55 years, which is younger than expected for the general population.Detailed analysis of causes of death correlated with clinical, laboratory, and tumor characteristics of patients in the 2 series allowed identification of a number of prognostic factors. Poor prognostic factors included higher fasting gastrin levels, presence of othe...

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Diagnosis of occult Zollinger-Ellison tumors by gastrin radioimmunoassay

Jaffe

Peskin

Kaplan

1972

Cancer

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The Zollinger‐Ellison syndrome can be best diagnosed by the measurement of elevated concentrations of peripheral venous gastrin. Using the gastrin radioimmunoassay, ulcerogenic tumors were diagnosed preoperatively in 4 patients. In three of these patients, no tumor could be found at laparotomy. In each, a tiny lesion was found in the submucosa of the duodenum, one at subsequent laparotomy, one in the duodenal stump resected for suspicion of retained antrum, and the third at autopsy. In the fourth patient, the duodenum was opened and a tiny duodenal islet cell adenoma resected. In the second and fourth patients, resection of the adenomas appears to have cured the patients without total gastrectomy; they are asymptomatic and have markedly lowered gastrin levels postoperatively. A plan for the management of duodenal islet cell tumors is presented which suggests that total gastrectomy might possibly be avoided if the surgeon is confident that the tumor is confined to the duodenum; this should be verified by following the course of the concentrations of peripheral venous gastrin.

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Zollinger-ellison syndrome with interesting variations

Cited by 27 publications

References 28 publications

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

Causes of Death and Prognostic Factors in Multiple Endocrine Neoplasia Type 1

Diagnosis of occult Zollinger-Ellison tumors by gastrin radioimmunoassay

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