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胆管内乳头状黏液性肿瘤:一种真正的疾病?

Intraductal papillary mucinous neoplasm of the biliary tract: a real disease?

出版信息

HPB (Oxford). 2009 Dec;11(8):684-91. doi: 10.1111/j.1477-2574.2009.00122.x.

Abstract

BACKGROUND

Despite increasing numbers of reports, biliary tract intraductal papillary mucinous neoplasm (BT-IPMN) is not yet recognized as a unique neoplasm. The aim of the present study was to define the presence of BT-IPMN in a large series of resected biliary neoplasms.

METHODS

From May 1994 to December 2006, BT-IPMN cases were identified by reviewing pathology specimens of all resected cholangiocarcinomas and other biliary neoplasms when cystic, papillary or mucinous features were cited in pathology reports.

RESULTS

BT-IPMN was identified in 23 out of 253 (9%) specimens using the strict histopathological criteria of IPMN. The most common presenting symptom was abdominal discomfort which was present in 15 patients (65%). Only one of the original operative pathology reports used the term IPMN; 16 (70%) used the terms cystic, mucinous and/or papillary. BT-IPMN was isolated to non-hilar extra-hepatic ducts in 12 (52%), intra-hepatic ducts in 6 (26%) and hilar extra-hepatic ducts in 5 patients (22%). Carcinoma was found in association with BT-IPMN in 19 patients (83%); 5-year survival was 38% after resection.

CONCLUSION

BT-IPMN occurs throughout the intra- and extra-hepatic biliary system and can be identified readily as a unique neoplasm. Broader acceptance of BT-IPMN as a unique neoplasm may lead to a better understanding of the pathogenesis of biliary malignancies.

摘要

背景

尽管报道的病例越来越多,但胆管内乳头状黏液性肿瘤(BT-IPMN)尚未被认为是一种独特的肿瘤。本研究的目的是在一系列大型切除的胆道肿瘤中确定 BT-IPMN 的存在。

方法

从 1994 年 5 月至 2006 年 12 月,通过回顾所有切除的胆管癌和其他胆道肿瘤的病理标本,当病理报告中提到囊性、乳头状或黏液性特征时,确定 BT-IPMN 病例。

结果

使用 IPMN 的严格组织病理学标准,在 253 个标本中确定了 23 个(9%)BT-IPMN。最常见的首发症状是腹部不适,15 例患者(65%)存在该症状。只有一份原始手术病理报告使用了 IPMN 一词;16 份(70%)使用了囊性、黏液性和/或乳头状术语。BT-IPMN 孤立于非肝门外胆管 12 例(52%),肝内胆管 6 例(26%),肝门外胆管 5 例(22%)。19 例(83%)患者发现与 BT-IPMN 相关的癌;切除后 5 年生存率为 38%。

结论

BT-IPMN 发生于肝内外胆管系统的各个部位,可作为一种独特的肿瘤轻易识别。更广泛地接受 BT-IPMN 作为一种独特的肿瘤,可能会更好地理解胆道恶性肿瘤的发病机制。

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