Intraductal neoplasms of the pancreas
Intraductal neoplasms of the pancreas
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DOI：
10.1053/j.semdp.2014.08.005
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发表时间：
2014-11-01
影响因子：
2.3
通讯作者：
Esposito, Irene
中科院分区：
文献类型：
--
作者：
Kloeppel, Guenter;Basturk, Olca;Esposito, Irene
有三种类型的胰腺肿瘤主要具有导管内生长模式：常见的，通常为囊性的导管内乳头状粘液性肿瘤（IPMN）;罕见的，通常为实性的导管内管状乳头状肿瘤（ITPN）;以及罕见的导管内管状幽门腺型腺瘤。除了这三种肿瘤类型外，通常具有实体生长模式的胰腺肿瘤，如腺泡细胞癌、神经内分泌肿瘤和未分化癌，尽管非常罕见，但也可能以导管内生长为主。IPMNs可细分为主管肿瘤和分支导管肿瘤;分为低度和高度发育不良组;以及具有肠、胰胆、嗜酸细胞或胃细胞分化的肿瘤。肠型、胰胆管型和嗜酸细胞型IPMN主要发生在胰头的主管道中，并且更常见地进展为浸润性腺癌。胃型IPMN通常是多灶性的，主要发生在钩突的分支导管中，并且进展为浸润性癌的风险较低。IPMN患者的预后在很大程度上取决于浸润性癌的亚型、存在和分期。ITPN是结节性肿瘤，通常位于胰头，由密集的管状腺体组成。分子遗传学揭示KRAS、GNAS和RNF 43是IPMN中最常突变的基因，而ITPN显示野生型KRAS。胰腺肿瘤基因测序和特定基因突变鉴定的最新进展也为未来开发胰腺导管内肿瘤新的基于基因的诊断测试提供了希望，这些测试甚至可能用于术前条件。(C)2014爱思唯尔公司All rights reserved.
There are three types of pancreatic neoplasms that predominantly have an intraductal growth pattern: the common, usually cystic, intraductal papillary mucinous neoplasms (IPMNs); the rare, usually solid intraductal tubulopapillary neoplasms (ITPNs); and the rare intraductal tubular pyloric gland-type adenoma. In addition to these three tumor types, pancreatic neoplasms with a usually solid growth pattern such as acinar cell carcinomas, neuroendocrine tumors, and undifferentiated carcinomas may present, though very rarely, as predominantly intraductally growing neoplasms. IPMNs can be subclassified into main duct and branch duct tumors; into low-and high-grade dysplasia groups; and into tumors with intestinal, pancreatobiliary, oncocytic, or gastric cellular differentiation. The intestinal-, pancreatobiliary-, and oncocytic-type IPMNs occur predominantly in the main duct of the head of the pancreas and more commonly progress to invasive adenocarcinomas. The gastric-type IPMNs are frequently multifocal, occur predominantly in the branch ducts of the uncinate process, and have a low risk of progressing to invasive carcinoma. The prognosis for patients with an IPMN depends largely on the subtype and the presence and the stage of an invasive carcinoma. ITPNs are nodular tumors, often in the pancreatic head, and composed of densely packed tubular glands. Molecular genetics reveal KRAS, GNAS, and RNF43 as the most frequently mutated genes in IPMNs, while ITPNs show wild-type KRAS. Recent progress in genetic sequencing of pancreatic neoplasms and the identificatiori of specific genetic mutations also holds promise for the future development of novel gene-based diagnostic tests in intraductal neoplasms of the pancreas that might even be used in preoperative conditions. (C) 2014 Elsevier Inc. All rights reserved.