Imamura Masayuki, Nakamoto Yuji, Uose Suguru, Komoto Izumi, Awane Masaaki, Taki Yoshiro
Neuroendocrine Tumor Center, Kansai Electric Power Hospital, 2-1-7 Fukushima, Fukushima, Osaka, 553-0003, Japan.
Department of Diagnostic Imaging and Nuclear Medicine, Kyoto University Graduate School of Medicine, Kyoto, Japan.
J Hepatobiliary Pancreat Sci. 2015 Aug;22(8):602-9. doi: 10.1002/jhbp.209. Epub 2015 Jan 26.
Functioning pancreaticoduodenal neuroendocrine tumors (PD-NETs) are popular in a textbook, but they are still unfamiliar to a general clinician, and delay of diagnosis or misdiagnosis has been reported even today. It is a consensus that sporadic functioning PD-NET is cured only by surgical resection. So, early detection and early resection is the gold standard for the treatment of functioning PD-NET. Functioning PD-NETs in patients with multiple endocrine neoplasia type 1 (MEN 1) are often multiple. You should check about MEN 1 whenever you encountered multiple PD-NET. They are diagnosed in younger age than sporadic cases. In most cases they are accompanied with numerous microscopic or macroscopic nonfunctioning P-NETs, which are potentially metastatic and the most common cause of death in MEN 1 patients.
功能性胰腺十二指肠神经内分泌肿瘤（PD-NETs）在教科书中常见，但普通临床医生对其仍不熟悉，甚至如今仍有诊断延迟或误诊的报道。目前的共识是，散发性功能性PD-NET仅通过手术切除才能治愈。因此，早期发现和早期切除是治疗功能性PD-NET的金标准。1型多发性内分泌腺瘤病（MEN 1）患者中的功能性PD-NET通常为多发。每当遇到多发PD-NET时，都应检查是否患有MEN 1。它们比散发性病例的诊断年龄更小。在大多数情况下，它们伴有大量微小或肉眼可见的无功能P-NET，这些肿瘤具有潜在转移性，是MEN 1患者最常见的死亡原因。
