Intraductal papillary mucinous neoplasms of the pancreas: An increasingly recognized clinicopathologic entity
Intraductal papillary mucinous neoplasms of the pancreas: An increasingly recognized clinicopathologic entity
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DOI：
10.1097/00000658-200109000-00005
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发表时间：
2001-09-01
影响因子：
9
通讯作者：
Lillemoe, KD
中科院分区：
文献类型：
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作者：
Sohn, TA;Yeo, CJ;Lillemoe, KD
目的探讨胰腺导管内乳头状粘液性肿瘤(IPMN)的诊治经验。背景资料：胰腺导管内乳头状粘液性肿瘤的发病率越来越高。结果13年间，60例胰腺浸润性导管腺癌患者行胰腺十二指肠切除术，其中40例近3年行胰腺切除术。发病时平均年龄为67.4+/-1.4岁。IPMNS患者最常见的症状是腹痛(59%)。大多数IPMN位于胰头或广泛累及胰腺腺体，其中70%经胰腺十二指肠切除术切除，22%经全胰腺切除，8%经远端胰腺切除术切除。22名患者(37%)有IPMNS合并浸润性腺癌。在免疫组织化学染色的一组IPMN中(n=50)，所有的导管内或非侵袭性成分都强烈表达DPC4，而相关的浸润性癌中84%表达DPC4。60例IPMN患者的5年生存率为57%。结论导管内乳头状黏液性肿瘤是一种独特的临床病理类型，其发生率不断提高。IPMN在临床、组织学和遗传学上都不同于胰腺导管腺癌。IPMNS独特的临床特征、原位或非侵袭期长以及患者良好的长期存活率为早期诊断、根治性切除以及进一步研究这些罕见肿瘤的分子遗传学和自然病史提供了独特的机会。
Objective To assess the authors' experience with intraductal papillary mucinous neoplasms of the pancreas (IPMNs).Summary Background Data Intraductal papillary mucinous neoplasms of the pancreas are being recognized with increasing frequency.Methods All patients who underwent pancreatic resection for an IPMN at the Johns Hopkins Hospital between January 1987 and December 2000 were studied. The data were compared with those of 702 concurrent patients with infiltrating ductal adenocarcinoma of the pancreas not associated with an IPMN resected by pancreaticoduodenectomy.Results In the 13-year time period, 60 patients underwent pancreatic resection for IPMNs, with 40 patients undergoing resection in the past 3 years. Mean age at presentation was 67.4 +/- 1.4 years. The most common presenting symptom in patients with IPMNs was abdominal pain (59%). Most IPMNs were in the head of the pancreas or diffusely involved the gland, with 70% being resected via pancreaticoduodenectomy, 22% via total pancreatectomy, and 8% via distal pancreatectomy. Twenty-two patients (37%) had IPMNs with an associated infiltrating adenocarcinoma. In a subset of IPMNs immunohistochemically stained for the Dpc4 protein (n = 50), all of the intraductal or noninvasive components strongly expressed Dpc4, whereas 84% of associated infiltrating cancers expressed Dpc4. The 5-year survival rate for all patients with IPMNs (n = 60) was 57%.Conclusion Intraductal papillary mucinous neoplasms represent a distinct clinicopathologic entity being recognized with increasing frequency. IPMNs are clinically, histologically, and genetically disparate from pancreatic ductal adenocarcinomas. The distinct clinical features, the presumably long in situ or noninvasive phase, and the good long-term survival of patients with IPMNs offer a unique opportunity for early diagnosis, curative resection, and further studies of the molecular genetics and natural history of these unusual neoplasms.