Xi Long-Fu, Jin Yun, Li Jiang-Tao
Department of Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Front Surg. 2022 Sep 1;9:963952. doi: 10.3389/fsurg.2022.963952. eCollection 2022.
As a rare disease, intrahepatic sarcomatoid cholangiocarcinoma (s-CCC) represents less than 1% of malignancies of the hepatobiliary system and its main clinical symptoms include abdominal pain and fever. Results of pathological examinations, despite being the "gold standard", can easily be confused with hepatocellular carcinoma (HCC). This report is about a 32-year-old male patient who was hospitalized due to occupancy of segment V of the liver for three days and had a history of chronic hepatitis B (CHB) over a 20-year span. Magnetic resonance imaging (MRI) showed a 43 mm × 52 mm-sized liver mass in the V segment, with patchy peripheral enhancement during the arterial phase and rapid wash-out during the portal and late phases. A laparoscopic hepatectomy of segment V, along with cholecystectomy, was performed. Histopathological and immunohistochemical examinations indicated a malignant neoplasm that was positive for vimentin and cytokeratin, with these features providing a positive diagnosis for intrahepatic sarcomatoid cholangiocarcinoma. After surgery, an adjuvant therapy of albumin-paclitaxel combined with gemcitabine regimen was given. No recurrence was found six months after the surgery, with follow-up still ongoing. This report aims to improve the awareness, diagnosis, and treatment of s-CCC.
肝内肉瘤样胆管癌（s-CCC）作为一种罕见疾病，在肝胆系统恶性肿瘤中所占比例不到1%，其主要临床症状包括腹痛和发热。尽管病理检查结果是“金标准”，但很容易与肝细胞癌（HCC）混淆。本报告介绍了一名32岁男性患者，因肝V段占位住院三天，有20年慢性乙型肝炎（CHB）病史。磁共振成像（MRI）显示V段有一个43 mm×52 mm大小的肝脏肿块，动脉期周边呈斑片状强化，门脉期和延迟期快速廓清。实施了V段腹腔镜肝切除术及胆囊切除术。组织病理学和免疫组织化学检查显示为一种波形蛋白和细胞角蛋白阳性的恶性肿瘤，这些特征确诊为肝内肉瘤样胆管癌。术后给予白蛋白紫杉醇联合吉西他滨方案的辅助治疗。术后六个月未发现复发，随访仍在进行中。本报告旨在提高对s-CCC的认识、诊断和治疗水平。
