Prevalence, characteristics and prognosis of MEN 1-associated glucagonomas, VIPomas, and somatostatinomas

Lévy-Bohbot, N.; Merle, Corinne; Goudet, P.; Delemer, Brigitte; Calender, Alain; Jolly, Damien; Thiéfin, Gérard; Cadiot, Guillaume

doi:10.1016/s0399-8320(04)95184-6

Cited by 135 publications

(96 citation statements)

References 14 publications

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“…Estimated 10-year survival rates of 23-62% have been reported (Levy-Bohbot et al 2004, Kouvaraki et al 2006, whereas this was 100% in a recent series (Lopez et al 2011). One has to keep in mind that with endoscopic ultrasound, more small NF-pNETs are currently diagnosed.…”

Section: Epidemiologymentioning

confidence: 95%

“…Four of these ten patients had liver metastases (40%). Ten-year survival was 54% (Levy-Bohbot et al 2004). …”

Section: Epidemiologymentioning

confidence: 96%

“…The largest combined experience comes from the French Endocrine Tumor Study Group, reporting on five glucagonomas, three VIPomas, and two somatostatinomas in MEN1, comprising 3.3% of the MEN1-related dpNETs (Levy-Bohbot et al 2004). Four of these ten patients had liver metastases (40%).…”

Section: Epidemiologymentioning

confidence: 99%

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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: Epidemiologymentioning

confidence: 95%

“…Four of these ten patients had liver metastases (40%). Ten-year survival was 54% (Levy-Bohbot et al 2004). …”

Section: Epidemiologymentioning

confidence: 96%

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

“…Pancreatic SOM-NETs have also been described in patients with MEN1 , Levy-Bohbot et al 2004, but an association of duodenal SOM-NETs with MEN1 and ZES caused by multiple duodenal gastrinomas has only recently been observed (Anlauf et al 2007). In contrast to the MEN1-associated duodenal gastrinomas, the MEN1-associated duodenal SOM-NETs have not so far been identified as a source of lymph node metastases.…”

Section: Endocrine-related Cancermentioning

confidence: 99%

Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity

Garbrecht¹,

Anlauf²,

Schmitt³

et al. 2008

Endocrine Related Cancer

166

128

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Somatostatin-producing neuroendocrine tumors (SOM-NETs) of the duodenum and pancreas appear to be heterogeneous. To determine their clinicopathological profiles, respective data were analyzed on a series of 82 duodenal and 541 pancreatic NETs. In addition, the clinical records of 821 patients with duodenal or pancreatic NETs were reviewed for evidence of a somatostatinoma syndrome. Predominant or exclusive expression of somatostatin was found in 21 (26%) duodenal and 21 (4%) pancreatic NETs. They were classified as sporadic (nZ31) or neurofibromatosis type 1 (NF1)-associated duodenal NETs (nZ3), gangliocytic paragangliomas (GCPGs; nZ6), or poorly differentiated neuroendocrine carcinomas (pdNECs; nZ2). In addition, five duodenal and four pancreatic SOM-NETs were found in five patients with multiple endocrine neoplasia type 1 (MEN1). Metastases occurred in 13 (43%) patients with sporadic or NF1-associated SOM-NETs, but in none of the duodenal or pancreatic MEN1-associated SOM-NETs or GCPGs. Sporadic advanced (stage IV) SOM-NETs were more commonly detected in the pancreas than in the duodenum. None of the patients (including the 821 patients for whom only the clinical records were reviewed) fulfilled the criteria of a somatostatinoma syndrome. Our data show that somatostatin expression is not only seen in sporadic NETs but may also occur in GCPGs, pdNECs, and hereditary NETs. Surgical treatment is effective in most duodenal and many pancreatic SOM-NETs. MEN1-associated SOM-NETs and GCPGs follow a benign course, while somatostatin-producing pdNECs are aggressive neoplasms. The occurrence of the so-called somatostatinoma syndrome appears to be extremely uncommon.

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“…A diffuse distribution throughout the pancreas or arising from the ectopic pancreas was rarely observed (Barahona-Garrido et al, 2009). In some reports, pancreatic somatostatinoma was detected in association with neurofibromatosis type 1, MEN-1, and von Hippel-Lindau syndromes (Maki et al, 1995;Levy-Bohbot et al, 2004;Barahona-Garrido et al, 2009). Recently, some clinicians presumed that there might exist an association between gastrointestinal stromal tumors (GIST) and somatostatinoma (Barahona-Garrido et al, 2009).…”

Section: Discussionmentioning

confidence: 99%

Pancreatic somatostatinoma with obscure inhibitory syndrome and mixed pathological pattern

Zhang

Xie

Gao

et al. 2010

J. Zhejiang Univ. Sci. B

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Somatostatinoma is a very rare neuroendocrine tumor that originates from D cells and accounts for less than 1% of all gastrointestinal endocrine tumors. The duodenum is the most frequent site for this tumor, followed by the pancreas. We here describe a 46-year-old Chinese woman who developed pancreatic somatostatinoma presenting with the characteristic "inhibitory" syndrome, but the symptoms were obscure and seemingly uncorrelated. This case is also unique for its large tumor size and mixed pathological pattern. Distal pancreatectomy was performed, and the patient has remained well since operation. As the syndromes of somatostatinoma may be obscure and atypical, clinicians should review all clinical findings to obtain an accurate diagnosis. Aggressive surgery is preferred to improve the survival.

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Prevalence, characteristics and prognosis of MEN 1-associated glucagonomas, VIPomas, and somatostatinomas

Cited by 135 publications

References 14 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

Somatostatin-producing neuroendocrine tumors of the duodenum and pancreas: incidence, types, biological behavior, association with inherited syndromes, and functional activity

Pancreatic somatostatinoma with obscure inhibitory syndrome and mixed pathological pattern

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