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胰腺癌肉瘤:全面的临床病理和分子特征分析。

Carcinosarcoma of the pancreas: comprehensive clinicopathological and molecular characterization.

机构信息

Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou, China; Zhejiang Provincial Key Laboratory of Pancreatic Disease, Hangzhou, China; Innovation Center for the Study of Pancreatic Diseases, Zhejiang, China.

Department of Pathology, The Second Affiliated Hospital of Zhejiang University, School of Medicine, Hangzhou, China.

出版信息

HPB (Oxford). 2020 Nov;22(11):1590-1595. doi: 10.1016/j.hpb.2020.01.017. Epub 2020 Feb 17.

Abstract

BACKGROUND

Carcinosarcoma of pancreas is a rare subtype of pancreatic cancer. The aim of this study was to comprehensively elaborate the clinicopathological and molecular features of this rare malignancy.

METHODS

Patients who were diagnosed with carcinosarcoma of the pancreas were retrospectively identified from pathology database of a single institution between 2012 and 2018.

RESULTS

A total of nine patients were identified. Pathological examination of tumor tissues from included patients showed coexisting carcinomatous and sarcomatous components. These two components were distinguished by mutually exclusive expression of cytokeratin and vimentin. The sarcomatous tissue exhibited more extensive proliferation, as revealed by Ki67 staining, and necrosis compared with the carcinomatous counterpart. Genomic analysis of tumor tissues for two patients demonstrated hotspot mutation at KRAS and TP53. Carcinomatous and sarcomatous components were separately obtained via laser captured microdissection in one patient, and mutations of driving genes were highly concordant between them. Besides, immunostaining of frequently-altered tumor suppressor genes for these two components suggested consistent expression patterns. The median overall survival for six patients with adequate follow-up was 14 months.

CONCLUSION

Carcinosarcoma of the pancreas represent a rare malignancy with distinct histological characteristics. Genomic and molecular analysis suggested monoclonal origin of carcinomatous and sarcomatous components.

摘要

背景

胰腺肉瘤样癌是一种罕见的胰腺癌亚型。本研究旨在全面阐述这种罕见恶性肿瘤的临床病理和分子特征。

方法

从 2012 年至 2018 年,我们从单一机构的病理数据库中回顾性地确定了诊断为胰腺肉瘤样癌的患者。

结果

共确定了 9 名患者。纳入患者的肿瘤组织的病理检查显示存在癌性和肉瘤性成分共存。这两种成分通过角蛋白和波形蛋白的相互排斥表达来区分。与癌性对应物相比,肉瘤性组织显示出更广泛的增殖,Ki67 染色和坏死。对两名患者的肿瘤组织进行基因组分析显示 KRAS 和 TP53 热点突变。在一名患者中通过激光捕获显微切割分别获得癌性和肉瘤性成分,驱动基因的突变在两者之间高度一致。此外,对这两种成分的常见肿瘤抑制基因进行免疫染色表明表达模式一致。在有足够随访的六名患者中,中位总生存期为 14 个月。

结论

胰腺肉瘤样癌是一种罕见的恶性肿瘤,具有独特的组织学特征。基因组和分子分析表明癌性和肉瘤性成分具有单克隆起源。

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