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

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

“…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.…”

“…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].…”

“…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.…”

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

“…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].…”

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

“…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.…”