ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms: Functional Pancreatic Endocrine Tumor Syndromes

Jensen, Robert T.; Cadiot, Guillaume; Brandi, Maria Luisa; Herder, Wouter W. de; Kaltsas, Gregory; Komminoth, Paul; Scoazec, Jean‐Yves; Salazar, Ramón; Sauvanet, Alain; Kianmanesh, Réza

doi:10.1159/000335591

Cited by 547 publications

(671 citation statements)

References 212 publications

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“…On the other hand, 20-25 % of gastrinomas and 4-5 % of insulinomas are reported to be complicated with MEN-1 [27]. The rate of MEN-1 association in functional PNETS in the present study (4.9 %) does not differ from that reported in Western nations [27,28]. However, MEN-1 associated with NF-PNETs was observed in only 4.0 % of cases in Japan.…”

Section: Discussioncontrasting

confidence: 64%

“…MEN-1 is reported to be complicated with NF-PNETs, gastrinoma, and insulinoma at frequencies of about 80 %, 50 %, and 20 %, respectively [23]. On the other hand, 20-25 % of gastrinomas and 4-5 % of insulinomas are reported to be complicated with MEN-1 [27]. The rate of MEN-1 association in functional PNETS in the present study (4.9 %) does not differ from that reported in Western nations [27,28].…”

Section: Discussionmentioning

confidence: 99%

“…Although surgical total excision is the standard treatment [37], some studies report that when radical treatment is difficult, debulking surgery of primary lesions and liver metastatic lesions effectively alleviate symptoms and improve prognosis [4,34,37]. On the other hand, in cases of unresectable advanced tumors, treatment aiming to improve prognosis by inhibiting tumor growth and improving clinical symptoms is necessary [8,13,27]. For this purpose, it is important to understand patient backgrounds, particularly epidemiological background, and be aware of the epidemiological differences between Japanese and Western populations.…”

Section: Discussionmentioning

confidence: 99%

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Epidemiological trends of pancreatic and gastrointestinal neuroendocrine tumors in Japan: a nationwide survey analysis

et al. 2014

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Background Although neuroendocrine tumors (NETs) are rare, the number of patients with NET is increasing. However, in Japan, there have been no epidemiological studies on NET since 2005; thus, the prevalence of NET remains unknown. Methods We reported the epidemiology of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) [pancreatic neuroendocrine tumors (PNETs) and gastrointestinal neuroendocrine tumors (GI-NETs)] in Japan in 2005. Here, we conducted the second nationwide survey on patients with GEP-NETs who received treatment in 2010. Results A total of 3,379 patients received treatment for PNETs in 2010, representing a 1.2-fold increase in the number of patients from 2005 to 2010. The prevalence was estimated to be 2.69/100,000, with an annual onset incidence of 1.27/100,000 in 2010. Non-functioning tumor (NF)-PNETs comprised 65.5 % of cases followed by insulinoma (20.9 %) and gastrinoma (8.2 %). Interestingly, the number of patients with NF-PNETs increased *1.8 fold since 2005. A total of 19.9 % of patients exhibited distant metastasis at initial diagnosis; 4.3 % had complications with multiple endocrine neoplasia type 1 (MEN-1), and only 4.0 % had NF-PNETs associated with MEN-1. Meanwhile, an estimated 8,088 patients received treatment for GI-NETs, representing a *1.8-fold increase since 2005. The prevalence was estimated to be 6.42/100,000, with an annual onset incidence of 3.51/100,000. The locations of GI-NETs varied: foregut, 26.1 %; midgut, 3.6 %; and hindgut, 70.3 %. Distant metastasis and complications with MEN-1 were observed in 6.0 and 0.42 % at initial diagnosis, respectively. The frequency of carcinoid syndrome in patients with GI-NETs was 3.2 %. Conclusion We clarified the epidemiological changes in GEP-NETs from 2005 to 2010 in Japan.

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Section: Discussioncontrasting

confidence: 64%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Epidemiological trends of pancreatic and gastrointestinal neuroendocrine tumors in Japan: a nationwide survey analysis

et al. 2014

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“…Hypoglycemia can then be ascribed to insulinoma if a spontaneous hypoglycemia is associated to concomitant insulin levels ≥6 µU/ml and C-peptide levels ≥ 200 pmol/l (or proinsulin levels ≥5 pmol/l), with the absence of sulfonylurea in the plasma or urine [6]. Alternatively, 72-h fasting test should be organized to document a fasting hypoglycemia with concomitant inappropriate elevated insulin and C-peptide and/or proinsulin levels [6].…”

Section: Discussionmentioning

confidence: 99%

Appendix insulin secreting neuroendocrine tumor in a diabetic patient: a challenging diagnosis.

Lombardi¹,

Ma²,

Grosso³

et al. 2017

JGPLD

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“…4 Surgical resection is the treatment of choice for patients with functional tumors, and it is preferred for most of the NFPNETs. [5][6][7] It is debatable whether all of the small and asymptomatic lesions should be routinely resected. 8 There is insufficient information about the natural history of NF-PNETs, especially when they are small.…”

Section: Introductionmentioning

confidence: 99%

Behavior of small, asymptomatic, nonfunctioning pancreatic neuroendocrine tumors (NF-PNETs)

Lee

Jung

2014

Pancreatology

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ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms: Functional Pancreatic Endocrine Tumor Syndromes

Cited by 547 publications

References 212 publications

Epidemiological trends of pancreatic and gastrointestinal neuroendocrine tumors in Japan: a nationwide survey analysis

Epidemiological trends of pancreatic and gastrointestinal neuroendocrine tumors in Japan: a nationwide survey analysis

Appendix insulin secreting neuroendocrine tumor in a diabetic patient: a challenging diagnosis.

Behavior of small, asymptomatic, nonfunctioning pancreatic neuroendocrine tumors (NF-PNETs)

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