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原发性肝肉瘤样癌:病例系列及文献综述

Primary Liver Sarcomatoid Carcinoma: A Case Series and Literature Review.

作者信息

Ji Wei, Xing Yunlong, Ma Jinshu, Zhao Zhuo, Xu Hongqin, Zheng Shuang, Li Wei, Li Xu

机构信息

Cancer Center, The First Hospital of Jilin University, Changchun, People's Republic of China.

Plastic Surgery, China-Japan Union Hospital, Jilin University, Changchun, People's Republic of China.

出版信息

J Hepatocell Carcinoma. 2021 Sep 7;8:1117-1127. doi: 10.2147/JHC.S325182. eCollection 2021.

Abstract

BACKGROUND

Primary liver sarcomatoid carcinoma (PLSC) is rare. To improve the understanding of PLSC, cases were described and reviewing the literature.

METHODS

A retrospective analysis was performed on 14 cases of PLSC diagnosed by pathology in Northeastern China from 2010 to 2020. An individual participant data analysis based on reported cases was conducted to determine epidemiological characteristics, clinical characteristics, and prognoses of PLSC.

RESULTS

A total of 136 cases involved our 14 cases and 122 cases from previous reports. The percentages of sarcomatoid hepatocellular carcinoma, sarcomatoid cholangiolocellular carcinoma, and mixed and unclassified types were 36.8%, 41.9%, 5.9%, and 15.4%, respectively. A total of 95.6% PLSC was found in Asia. There was a lower percentage of hepatitis-infected patients in Japan, when compared with the Republic of Korea (38.5% vs 70.0%, P<0.05). Five cases were initially misdiagnosed as a hepatic abscess by imaging. A total of 36.7% cases had metastases when being diagnosed, and 68.9% cases relapsed during the follow-up. The median disease-free survival and overall survival (OS) were 3 months and 5 months, respectively. Only radical surgery (hazard ratio = 0.308, 95% confidence interval 0.186-0.512, P<0.001) indicated a better OS.

CONCLUSION

PLSC was more prevalent in Asia and there were possibilities of misdiagnoses. Surgery is still an effective treatment and can significantly prolong the OS. Only limited strategies for recurrent or advanced PLSC, immunotherapy may be possible treatment.

摘要

背景

原发性肝脏肉瘤样癌(PLSC)较为罕见。为提高对PLSC的认识,现将病例进行描述并复习文献。

方法

对2010年至2020年在中国东北地区经病理诊断的14例PLSC进行回顾性分析。基于报告病例进行个体参与者数据分析,以确定PLSC的流行病学特征、临床特征和预后。

结果

共纳入14例病例及之前报告的122例病例,总计136例。肉瘤样肝细胞癌、肉瘤样胆管细胞癌、混合型及未分类型的比例分别为36.8%、41.9%、5.9%和15.4%。95.6%的PLSC病例来自亚洲。与韩国相比,日本的肝炎感染患者比例较低(38.5%对70.0%,P<0.05)。5例患者最初经影像学检查被误诊为肝脓肿。36.7%的病例在诊断时已有转移,68.9%的病例在随访期间复发。无病生存期和总生存期(OS)的中位数分别为3个月和5个月。只有根治性手术(风险比=0.308,95%置信区间0.186 - 0.512,P<0.001)显示OS较好。

结论

PLSC在亚洲更为常见,存在误诊的可能性。手术仍然是一种有效的治疗方法,可显著延长OS。对于复发或晚期PLSC只有有限的治疗策略,免疫治疗可能是一种治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4caf/8434859/0467d416cc6a/JHC-8-1117-g0001.jpg

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