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.



中文翻译：

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原发性肝肉瘤样癌：病例系列和文献回顾

背景：原发性肝肉瘤样癌（PLSC）很少见。为了提高对 PLSC 的理解，对案例进行了描述并回顾了文献。
方法：对2010-2020年东北地区14例病理确诊PLSC进行回顾性分析，根据报告病例进行个体参与数据分析，确定PLSC的流行病学特征、临床特征及预后。
结果：共有136个案例涉及我们的14个案例和之前报告的122个案例。肉瘤样肝细胞癌、肉瘤样胆管细胞癌、混合型和未分类型的百分比分别为36.8%、41.9%、5.9%和15.4%。在亚洲发现了 95.6% 的 PLSC。与韩国相比，日本的肝炎感染患者比例较低（38.5% vs 70.0%，P<0.05）。5例最初被影像学误诊为肝脓肿。确诊时有36.7%的病例有转移，随访时有68.9%的病例复发。中位无病生存期和总生存期 (OS) 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）表明 OS 更好。
结论： PLSC在亚洲更为普遍，存在误诊的可能。手术仍然是一种有效的治疗方法，可以显着延长 OS。只有有限的复发或晚期 PLSC 策略，免疫治疗可能是可能的治疗方法。