Intraductal Tubulopapillary Neoplasms of the Pancreas Distinct From Pancreatic Intraepithelial Neoplasia and Intraductal Papillary Mucinous Neoplasms
Intraductal Tubulopapillary Neoplasms of the Pancreas Distinct From Pancreatic Intraepithelial Neoplasia and Intraductal Papillary Mucinous Neoplasms
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DOI：
10.1097/pas.0b013e3181a162e5
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发表时间：
2009-08-01
影响因子：
5.6
通讯作者：
Furukawa, Toru
中科院分区：
文献类型：
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作者：
Yamaguchi, Hiroshi;Shimizu, Michio;Furukawa, Toru
我们遇到过一些不寻常的导管内胰腺肿瘤，主要是管状乳头状生长。我们收集了10例类似的“导管内管状乳头状肿瘤（ITPNs）”的资料，并分析了其临床病理和分子特征。肿瘤标本男5例，女5例，平均年龄58岁。ITPNs为实性结节状肿瘤，阻塞扩张的胰管，不含任何可见的粘蛋白。肿瘤细胞呈管状乳头状，胞浆黏蛋白含量极少。肿瘤表现出一致的高度异型性。坏死灶多见，部分病例可见浸润。itpn细胞角蛋白7和/或角蛋白19免疫组化阳性，胰蛋白酶、MUC2、MUC5AC和筋膜蛋白免疫组化阴性。分子研究发现1例TP53、SMAD4异常表达，P-catenin未见异常表达。8例患者未见KRAS和BRAF突变。8例患者存活，无复发，1例患者死于肝转移，1例患者存活，但有复发，并接受了额外的胰腺切除术。有丝分裂计数和Ki-67标记指数与侵袭有显著相关性。ITPN的所有特征都不同于其他已知的导管内胰腺肿瘤，包括胰腺上皮内瘤变、导管内乳头状粘液瘤和导管内腺泡细胞癌。除了管状乳头状生长模式外，导管内管状癌表现出与ITPN相似的几个特征。总之，itpn可以被认为是一种新的疾病实体，包括导管内小管癌作为一种形态变异。
We have encountered cases of unusual intraductal pancreatic neoplasms with predominant tubulopapillary growth. We collected data on 10 similar cases of "intraductal tubulopapillary neoplasms (ITPNs)" and analyzed their clinicopathologic and molecular features. Tumor specimens were obtained from 5 men and 5 women with a mean age of 58 years. ITPNs were solid and nodular tumors obstructing dilated pancreatic ducts and did not contain any visible mucin. The tumor cells formed tubulopapillae and contained little cytoplasmic mucin. The tumors exhibited uniform high-grade atypia. Necrotic foci were frequently observed, and invasion was observed in some cases. The ITPNs were immunohistochemically positive for cytokeratin 7 and/or cytokeratin 19 and negative for trypsin, MUC2, MUC5AC, and fascin. Molecular studies revealed abnormal expressions of TP53 and SMAD4 in 1 case, but aberrant expression of P-catenin was not observed. No mutations in KRAS and BRAF were observed in the 8 cases that were examined. Eight patients are alive without recurrence, I patient died of liver metastases, and I patient is alive but had a recurrence and underwent additional pancreatectomy. The mitotic count and Ki-67 labeling index were significantly associated with invasion. All the features of ITPN were distinct from those of other known intraductal pancreatic neoplasms, including pancreatic intraepithelial neoplasia, intraductal papillary mucinous neoplasm, and the intraductal variant of acinar cell carcinoma. Intraductal tubular carcinomas showed several features that were similar to, those of ITPN, except for the tubulopapillary growth pattern. In conclusion, ITPNs can be considered to represent a new disease entity encompassing intraductal tubular carcinoma as a morphologic variant.