The Influence of Total Castrectomy on Survival in Malignant Zollinger- Ellison Tumors

Fox, Paul S.; Hofmann, James W.; DeCosse, Jerome J.; Wilson, Stuart D.

doi:10.1097/00000658-197410000-00020

Cited by 93 publications

(7 citation statements)

References 11 publications

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“…In various studies of patients with ZES a number of specific disease related features were reported to have prognostic significance including disease duration 476 , extent of hypergastrinemia 191,292,397,405,459,476,484 , basal acid output 191,476 , the presence of peptic ulcer disease or its complications 108,117,191,199,200,483 , previous gastric acid reducing surgery 476 and antisecretory drug used 476 . Each of these was compared in MEN1/ZES patients that were alive or that died during follow-up (Table 4).…”

Section: Results (Tables 1–13)mentioning

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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Section: Results (Tables 1–13)mentioning

confidence: 99%

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

et al. 2013

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“…Before the introduction of H2-receptor antagonists [20] and PPIs [21], surgery was primarily aimed at the control of gastric acid hypersecretion symptoms and prevention of its sequelae, mainly through a total gastrectomy, vagotomy or, when possible, tumour resection [22,23]. The ability of medical therapy to effectively manage this syndrome has shifted the role of surgery on the oncological disease itself and therefore onto its ability to identify and resect the tumour and eventually its metastases [23][24][25][26][27][28][29][30].…”

Section: Surgical Treatmentmentioning

confidence: 99%

The Zollinger-Ellison syndrome: is there a role for somatostatin analogues in the treatment of the gastrinoma?

et al. 2017

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The high sstr-expression in gastrinomas make them highly responsive to SSAs and support the use of such drugs to counteract the tumour growth in patients not amenable to surgical cure. Unfortunately, limited data, mainly case reports or small series, support the use of SSAs in advanced gastrinomas, therefore, it is difficult to quantify their ability to control tumour growth and disease progression.

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“…Initially, total gastrectomy was the only form of effective antisecretory therapy [15,28,29,[74][75][76], however, recent studies demonstrated that, with the availability of potent antisecretory agents such as omeprazole, gastric acid hypersecretion can now be easily controlled medically in all patients with Zollinger-Ellison syndrome [1,4,[69][70][71]. Therefore, it is currently generally recommended, except for the patient who cannot or will not take oral medication, that total gastrectomy is not indicated[l, 3,4,104].…”

Section: Is There Still a Role For Gastric Surgery In Patients With Z...mentioning

confidence: 99%

Unresolved surgical issues in the management of patients with Zollinger‐Ellison syndrome

Norton

Jensen

1991

World j. surg.

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In the last 10 years, there have been a number of major advances that have markedly changed the management of patients with Zollinger-Ellison syndrome. These advances have been described recently in a number of excellent reviews. After reading each of these reviews, including our own, one is left generally with the impression that most of the important problems and particularly those involving surgery in the treatment of this disease have been resolved; however, in considering the possible problems to address in protocols in the coming years in our patients with Zollinger-Ellison syndrome followed at the National Institutes of Health, it became apparent to us that not only will surgery play an increasingly important role in the management of this disease, but that there are a number of major questions in which various aspects involving surgery are not resolved. Furthermore, resolution of a number of these areas will have applicability to the surgical treatment of other islet cell tumors. The purpose of this article is to call attention to these questions with the hope that other investigators may also consider these and specifically attempt to address some of these issues in the surgical treatment of Zollinger-Ellison syndrome in prospective studies.

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The Influence of Total Castrectomy on Survival in Malignant Zollinger- Ellison Tumors

Cited by 93 publications

References 11 publications

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

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

The Zollinger-Ellison syndrome: is there a role for somatostatin analogues in the treatment of the gastrinoma?

Unresolved surgical issues in the management of patients with Zollinger‐Ellison syndrome

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