Importance of Surveillance for Multiple Endocrine Neoplasia-1 and Surgery in Patients With Sporadic Zollinger–Ellison Syndrome

Singh, Maneesh H.; Fraker, Douglas L.; Metz, David C.

doi:10.1016/j.cgh.2012.08.014

Cited by 14 publications

(17 citation statements)

References 40 publications

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“…In contrast, in one series a survival benefit was found for MEN1 patients, but separate baseline characteristics were not provided (Melvin et al 1993). Another series also found survival benefit in MEN1, with no significant baseline differences in liver metastases (MEN1 6% vs sporadic 24%, PZ0.24), but this might be due to the small number of patients and selection or referral bias cannot be excluded (Singh et al 2012). Overall, the available data seem to point to a similar natural course for MEN1-related and sporadic gastrinoma.…”

Section: Endocrine-related Cancermentioning

confidence: 59%

“…In MEN1-related gastrinoma, synchronous lymph node metastases are reported in 45-69% (Ruszniewski et al 1993, Thompson 1995, Weber et al 1995, Jensen 1998, Norton et al 1999, Imamura et al 2011, Singh et al 2012, Ito et al 2013, with two series reporting 23-35% (Thompson 1998, Cadiot et al 1999 and two series reporting 80%. (Dickson et al 2011, Lopez et al 2013.…”

Section: Epidemiologymentioning

confidence: 99%

“…MEN1-related dpNETs are seen one to two decades earlier than their sporadic counterparts (Jensen 1998, Nikfarjam et al 2008, Anlauf et al 2009, Crippa et al 2012, Singh et al 2012. Insulinomas have the lowest age of onset (patients are usually in their twenties to thirties at diagnosis), patients with gastrinomas and NF-pNETs are usually diagnosed in their thirties (Jensen 1998, Cougard et al 2000, Triponez et al 2006a, Sakurai et al 2012b.…”

Section: Epidemiologymentioning

confidence: 99%

See 2 more Smart Citations

Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis

Pieterman¹,

Conemans²,

Dreijerink³

et al. 2014

Endocrine-Related Cancer

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Mutations of the multiple endocrine neoplasia type 1 (MEN1) gene lead to loss of function of its protein product menin. In keeping with its tumor suppressor function in endocrine tissues, the majority of the MEN1-related neuroendocrine tumors (NETs) show loss of heterozygosity (LOH) on chromosome 11q13. In sporadic NETs, MEN1 mutations and LOH are also reported, indicating common pathways in tumor development. Prevalence of thymic NETs (thNETs) and pulmonary carcinoids in MEN1 patients is 2-8%. Pulmonary carcinoids may be underreported and research on natural history is limited, but disease-related mortality is low. thNETs have a high mortality rate. Duodenopancreatic NETs (dpNETs) are multiple, almost universally found at pathology, and associated with precursor lesions. Gastrinomas are usually located in the duodenal submucosa while other dpNETs are predominantly pancreatic. dpNETs are an important determinant of MEN1-related survival, with an estimated 10-year survival of 75%. Survival differs between subtypes and apart from tumor size there are no known prognostic factors. Natural history of nonfunctioning pancreatic NETs needs to be redefined because of increased detection of small tumors. MEN1-related gastrinomas seem to behave similar to their sporadic counterparts, while insulinomas seem to be more aggressive. Investigations into the molecular functions of menin have led to new insights into MEN1-related tumorigenesis. Menin is involved in gene transcription, both as an activator and repressor. It is part of chromatin-modifying protein complexes, indicating involvement of epigenetic pathways in MEN1-related NET development. Future basic and translational research aimed at NETs in large unbiased cohorts will clarify the role of menin in NET tumorigenesis and might lead to new therapeutic options.

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Section: Endocrine-related Cancermentioning

confidence: 59%

Section: Epidemiologymentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

See 1 more Smart Citation

Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis

Pieterman¹,

Conemans²,

Dreijerink³

et al. 2014

Endocrine-Related Cancer

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show abstract

“…Compared with sporadic NETs, in MEN1, we can observe the early development of functioning syndromes such as ZES, because patients with MEN1 are young. Patients with ZES and MEN1 are on average 10 years younger than those with sporadic ZES at the time of diagnosis (Jensen et al 2008, Singh et al 2012. Therefore, ZES should be considered in patients with MEN1 with new digestive symptoms, including loose stools, especially when diarrhea promptly disappears when using proton pump inhibitor (PPI) therapy, epigastric pain and signs of gastroesophageal reflux even without severe esophagitis or tiny duodenal erosions on gastroscopy.…”

Section: Diagnosis Of Zesmentioning

confidence: 99%

“…Even in sporadic ZES, the demonstration of a reduction in liver metastases development relies on very few data obtained in noncomparative studies (Fraker et al 1994). In patients with ZES/MEN1, surgery was not associated with a decrease in metastases (Cadiot et al 1999, Jensen et al 2008, Singh et al 2012). This could be: 1-because the risk of metastases is late and low, requiring a very long follow-up to be able to show differences in the risk; 2-because patients underwent surgery too late to reduce metastases development.…”

Section: Peculiarities Of Zes Treatment In Patients With Men1mentioning

confidence: 99%

The future: surgical advances in MEN1 therapeutic approaches and management strategies

Sadowski¹,

Cadiot²,

Dansin³

et al. 2017

Endocrine-Related Cancer

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Multiple endocrine neoplasia type 1 (MEN1) is a hereditary autosomal dominant disorder associated with numerous neuroendocrine tumors (NETs). Recent advances in the management of MEN1 have led to a decrease in mortality due to excess hormones; however, they have also led to an increase in mortality from malignancy, particularly NETs. The main challenges are to localize these tumors, to select those that need therapy because of the risk of aggressive behavior and to select the appropriate therapy associated with minimal morbidity. This must be applied to a hereditary disease with a high risk of recurrence. The overall aim of management in MEN1 is to ensure that the patient remains disease-and symptom-free for as long as possible and maintains a good quality of life. Herein, we review the changes that occurred in the last 20 years in the surgical management of MEN1-associated functional and non-functional pancreaticoduodenal NETs and thymic and bronchial NETs.

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Strategies to improve the outcome of emergency surgery for perforated peptic ulcer

Søreide

Thorsen

Søreide

2014

Journal of British Surgery

179

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Importance of Surveillance for Multiple Endocrine Neoplasia-1 and Surgery in Patients With Sporadic Zollinger–Ellison Syndrome

Cited by 14 publications

References 40 publications

Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis

Thoracic and duodenopancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: natural history and function of menin in tumorigenesis

The future: surgical advances in MEN1 therapeutic approaches and management strategies

Strategies to improve the outcome of emergency surgery for perforated peptic ulcer

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