Zollinger-Ellison Syndrome in a Patient with Secondary Amenorrhea; Report of a Case with Fatal Outcome

Neef, J.; Krull, G.H.; Vonk, Jan; A, Vos; Bool, J.

doi:10.1016/s0015-0282(16)37267-3

Cited by 4 publications

(6 citation statements)

References 32 publications

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“…1). In no patient was death due to the acute complications of peptic ulcer disease due to uncontrolled gastric acid hypersecretion as was commonly reported in the past 15,43,72,73,83,88,158,174,180,224,225,240,254,307,353,366,386,399,438,444,458,461,465,466 , demonstrating the effectiveness of long-term medical management of the acid hypersecretion as reported in a number of studies 69,123,136,172,192,201,268,278,279,284,287,350 , because no patient underwent a total gastrectomy during follow-up at NIH for control of the gastric acid hypersecretion. In the NIH patients all the MEN1-related deaths were due to a neuroendocrine tumor (NET) in some manner (carcinoid, PET, other endocrine tumor) and in 79% it was due to the malignant nature of the NET.…”

Section: Results (Tables 1–13)mentioning

confidence: 91%

“…First, most studies in the literature are retrospective, many contain only small numbers of cases limiting analyses, and most of the larger studies contained pooled data from different centers leading to data variation and frequent use of historical records or retrospective data to determine causes of death and therefore have limitations. Second, in various studies, in which 20–70 % (mean 54%-18 series) of the MEN1 patients developed Zollinger-Ellison syndrome 15,49,63,73,102,152,189,208,212,261,264,298,348,387,418,436,436,444 , the most common functional PET seen in patients with MEN1 140,195,261,418 , the uncontrolled gastric acid hypersecretion, which is characteristic of ZES 200,201,279,364 , was a leading cause of early death 15,43,72,73,83,88,158,180,224,225,240,254,307,353,366,386,399,438,444,458,461,465,466 . Gastric acid hypersecretion accounted for up to 73–91% of all deaths in some early series 15,195,224,254,444 .…”

Section: Discussionmentioning

confidence: 99%

“…At present PPIs are the drugs of choice controlling the acid hypersecretion in almost every ZES patient, both acutely and long-term 123,171,189,192,227,266,270,278,281,284,295,350 , because tachyphylaxis does not develop 123,171,189,192,227,270,278,281,284 . The availability of PPIs has almost completely eliminated the lethal complications of peptic ulcer disease (perforation, bleeding, penetration) in MEN1/ZES patients which were so frequent in older studies 15,17,43,72,73,83,88,107,129,140,158,180,180,189,195,224,225,240,254,307,310,353,366,386,399,438,444,458,461,462,465,466,474 , and therefore their use has changed the current natural history of MEN1 patients in regard to times and causes of death. At present there are no prospective studies of MEN1 patient’s natural history and causes of death that reflect these changes in the control of acid hypersecretion.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, at present death uncommonly is death due to complications of gastric acid hypersecretion which was so frequent in the past 15,43,72,73,83,88,158,180,224,225,240,254,307,353,366,386,399,438,444,458,461,465,466 , due to hormone hypersecretion by other PETs such as insulinomas 15,306,334,375,466 ; due to the complications of untreated hyperparathyroidism such as renal failure or hypercalcemic crises 15,32,68,88,129,224,359,427,438,440,444,461,465,466 ; due to untreated pituitary disease (apoplexy, hormone excess state, etc) 222,447 or to other uncommon hormone excess states (ectopic Cushing’s from adrenal tumors, other functional PETs, etc) 165,228,447,468,475 .…”

Section: Conclusion and Summary Of Important Points (Table 14)mentioning

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%

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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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