Long-term Follow-up of Patients with Multiple Endocrine Neoplasia Type 1

Sakurai, Akihiro; Katai, Miyuki; Yamashita, Koh; Mori, Junichiro; Fukushima, Yoshimitsu; Hashizume, Kiyoshi

doi:10.1507/endocrj.k06-147

Cited by 26 publications

(33 citation statements)

References 38 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…One has to keep in mind that with endoscopic ultrasound, more small NF-pNETs are currently diagnosed. They are usually indolent and demonstrate slow growth, with a doubling time of 5-10 years (Kann et al 2006, Sakurai et al 2007. When 46 patients with NF-pNETs !2 cm without surgical treatment were followed over 10 years, 17% showed increase in size, 11% developed a functional syndrome, 65% displayed stable disease, 2% died due to metastatic NF-pNETs, 2% due to other causes, and 2% was lost to follow-up (Triponez F, Goudet P, AFCE, & GTE unpublished observations presented at ENETS 2013, Barcelona, Spain).…”

Section: Epidemiologymentioning

confidence: 99%

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

View full text Add to dashboard Cite

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.

show abstract

Section: Epidemiologymentioning

confidence: 99%

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

View full text Add to dashboard Cite

show abstract

“…Most cohorts report that thymic NET occur predominantly in men with a mean age around the fifth decade (Teh et al 1997, Gibril et al 2003, Ferolla et al 2005, Sakurai et al 2007, Habbe et al 2008, Goudet et al 2009, de Laat et al 2014 in contrast with previous studies, a Japanese study reported a relatively high percentage (36%) of women in their cohort of MEN1 patients with a thymic NET (Sakurai et al 2013).…”

Section: Thymic Neuroendocrine Tumorsmentioning

confidence: 74%

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

Leeuwaarde

Laat

Pieterman

et al. 2017

Endocrine-Related Cancer

View full text Add to dashboard Cite

Multiple endocrine neoplasia type 1 is a rare autosomal inherited disorder associated with a high risk for patients to simultaneously develop tumors of the parathyroid glands, duodenopancreatic neuroendocrine tumors and tumors of the anterior pituitary gland. Early identification of MEN1 in patients enables presymptomatic screening of manifestations, which makes timely interventions possible with the intention to prevent morbidity and mortality. Causes of death nowadays have shifted toward local or metastatic progression of malignant neuroendocrine tumors. In early cohorts, complications like peptic ulcers in gastrinoma, renal failure in hyperparathyroidism, hypoglycemia and acute hypercalcemia were the primary causes of early mortality. Improved medical treatments of these complications led to a significantly improved life expectancy. The MEN1 landscape is still evolving, considering the finding of breast cancer as a new MEN1-related manifestation and ongoing publications on follow-up and medical care for patients with MEN1. This review aims at summarizing the most recent insights into the follow-up and medical care for patients with MEN1 and identifying the gaps for future research.

show abstract

“…While there is consensus that all NFPNETs larger than 2 cm or associated with symptoms (e.g., pain) should be resected due to the increased risk of malignancy and metastases, the optimal surgical approach for asymptomatic NF-PNETs less than 2 cm is less clear. 7,37,38 In patients with MEN-1, NF-PNETs are multifocal and according to autopsy series are present in 80% of patients with this syndrome. 7 Due to the multiplicity of tumors in these patients, local resection is not expected to result in a cure, and MEN-1 patients with NF-PNETs less than 2 cm have been found to have a survival of 100% at 15 years and rarely develop advanced disease.…”

Section: Nf-pnets Associated With Hereditary Syndromesmentioning

confidence: 99%

Contemporary Management of Nonfunctioning Pancreatic Neuroendocrine Tumors

Minter

Simeone

2012

Journal of Gastrointestinal Surgery

View full text Add to dashboard Cite

In recent years the detection of nonfunctioning pancreatic neuroendocrine tumors has increased coincident with the increased use of cross-sectional imaging and is now thought to represent 2-10% of all pancreatic tumors. Improved understanding of the biology and behavior of these heterogeneous tumors is needed to guide therapy. The present review discusses diagnostics, therapeutics including emerging molecular analytics, and surveillance recommendations.

show abstract

Long-term Follow-up of Patients with Multiple Endocrine Neoplasia Type 1

Cited by 26 publications

References 38 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: medical advances in MEN1 therapeutic approaches and management strategies

Contemporary Management of Nonfunctioning Pancreatic Neuroendocrine Tumors

Contact Info

Product

Resources

About