伴有导管囊性扩张的胰腺肿瘤:导管内乳头状黏液性肿瘤和导管内嗜酸性乳头状肿瘤。

Pancreatic tumors with cystic dilatation of the ducts: intraductal papillary mucinous neoplasms and intraductal oncocytic papillary neoplasms.

作者信息

Adsay N V, Longnecker D S, Klimstra D S

机构信息

Department of Pathology, The Karmanos Cancer Institute, Harper Hospital, Wayne State University School of Medicine, Detroit, MI 48201, USA.

出版信息

Semin Diagn Pathol. 2000 Feb;17(1):16-30.

DOI:
Abstract

Intraductal papillary mucinous neoplasms (IPMNs) and intraductal oncocytic papillary neoplasms (IOPNs) are the 2 types of intraductal neoplasms of the pancreas that may appear cystic because of dilatation of the ducts. Both are characterized by intraductal proliferation of mucinous cells usually arranged in papillary patterns. This proliferation is often associated with intraluminal mucin accumulation, which produces cystic dilatation of the ducts, mimicking mucinous cystic neoplasms. Endoscopic and radiologic studies and careful macroscopic examination are crucial for the correct diagnosis of IPMNs and IOPNs by showing the origin within the native ducts. Microscopically, these tumors display a spectrum of cytoarchitectural atypia that ranges from adenoma to borderline and to carcinoma-in-situ. Although they are defined as "intraductal tumors," IPMNs and IOPNs are associated with invasive carcinoma in about a third of the cases. It, therefore, appears that, like mucinous cystic neoplasms or pancreatic intraepithelial neoplasia involving the smaller ducts associated with ordinary ductal adenocarcinomas, these tumors are precursors of invasive carcinoma. Invasive carcinomas associated with IPMNs are of either tubular or colloid (mucinous noncystic) types, whereas those associated with IOPNs may be oncocytic. Even in the presence of invasive carcinoma, these tumors may follow a more protracted clinical course than ordinary ductal adenocarcinoma. On the other hand, rare examples of IPMNs after an aggressive clinical course despite the lack of any identifiable invasive carcinoma are on record. Therefore, IPMNs and IOPNs should be examined carefully and sampled extensively, first, to confirm that the main pathology is an intraductal process and, more importantly, to rule out the presence of an invasive carcinoma.

摘要

导管内乳头状黏液性肿瘤(IPMNs)和导管内嗜酸性乳头状肿瘤(IOPNs)是胰腺导管内肿瘤的两种类型,由于导管扩张,它们可能呈囊性。两者的特征均为黏液细胞在导管内呈乳头状排列的增殖。这种增殖常伴有管腔内黏液积聚,导致导管囊性扩张,类似黏液性囊性肿瘤。内镜和放射学检查以及仔细的大体检查对于IPMNs和IOPNs的正确诊断至关重要,因为它们能显示肿瘤起源于固有导管。显微镜下,这些肿瘤表现出一系列细胞结构异型性,从腺瘤到交界性病变再到原位癌。尽管它们被定义为“导管内肿瘤”,但IPMNs和IOPNs在约三分之一的病例中与浸润性癌相关。因此,似乎与黏液性囊性肿瘤或与普通导管腺癌相关的累及较小导管的胰腺上皮内瘤变一样,这些肿瘤是浸润性癌的前驱病变。与IPMNs相关的浸润性癌为管状或胶样(黏液性非囊性)类型,而与IOPNs相关的浸润性癌可能为嗜酸性。即使存在浸润性癌,这些肿瘤的临床病程可能比普通导管腺癌更为迁延。另一方面,有记录显示,尽管缺乏任何可识别的浸润性癌,但仍有罕见的IPMNs病例具有侵袭性临床病程。因此,应对IPMNs和IOPNs进行仔细检查并广泛取材,首先要确认主要病理为导管内病变,更重要的是排除浸润性癌的存在。

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