三种预后较差少见病理类型原发性肝癌的临床特点和预后

Clinical characteristics and prognosis of three rare and poor-prognostic subtypes of primary liver carcinoma

目的研究三种预后较差的少见病理类型原发性肝癌的临床特点和预后相关因素。方法回顾性分析1998年10月至2013年6月收治的经术后病理证实的69例少见病理类型原发性肝癌（肝细胞-胆管细胞混合型肝癌34例，巨细胞型肝癌19例，肉瘤样肝癌16例）患者的临床资料，并与同期收治的80例低分化肝细胞肝癌患者进行比较。结果4组不同类型原发性肝癌患者的性别、年龄、乙肝表面抗原（HBsAg）阳性率、甲胎蛋白水平、肝硬化情况、肿瘤部位、肿瘤大小以及肝切除术式的比较，差异均无统计学意义（均P〉0．05）；但术前癌胚抗原水平、肿瘤数目、是否行肝门淋巴结清扫术、有无邻近器官侵犯、有无脉管癌栓的差异均有统计学意义（均P〈0．05）。生存分析的结果显示，肝细胞-胆管细胞混合型肝癌、巨细胞型肝癌、肉瘤样肝癌和低分化肝细胞肝癌组患者的中位生存时间分别为20、13、8和36个月。肝细胞一胆管细胞混合型肝癌组患者的1、3、5年生存率分别为61．8％、29．4％和20．，6％，巨细胞型肝癌组患者的1、3、5年生存率分别为52．6％、31．6％和0％，肉瘤样肝癌组患者的1、3、5年生存率分别为31．3％、0％和0％，低分化肝细胞肝癌组患者的1、3、5年生存率分别为83．8％、33．8％和7．5％，肉瘤样肝癌组患者的预后最差（P〈0．05）。Cox多因素分析的结果显示，切缘阳性为影响肝细胞一胆管细胞混合型肝癌、巨细胞型肝癌和肉瘤样肝癌患者预后的独立危险因素。结论肝细胞一胆管细胞混合型肝癌、巨细胞型肝癌和肉瘤样肝癌是三种预后较差的肝癌病理类型，尤以肉瘤样肝癌恶性程度最高，预后最差，手术应尽可能行根治性切除。

Objective To explore the clinicopathological features and prognostic factors of three rare and poor-prognostic pathological subtypes of primary liver carcinoma, and improve the clinical diagnosis and surgical treatment. Methods A retrospective analysis of clinicopathological data of 69 patients with rare pathological subtypes of primary liver carcinoma, diagnosed by postoperative pathology in our hospital from October 1998 to June 2013 was carried out. The data of 80 cases of common poorly differentiated hepatoeellular carcinoma treated in the same period were collected as control group. Kaplan-Meier method was used to analyze the survival rate, and Cox proportional hazards model was used for prognostic analysis in the patients. Results Thirty-four cases were combined hepatocellular carcinoma and cholangiocarcinoma （CCC, 28 males, 6 females）, with a median age of 52 years （range, 33 to 73）. Ninteen cases were giant cell carcinoma （GCC, 16 males and 3 females）, with a median age of 59 years （range, 38 to 66）. Sixteen cases were sareomatoid carcinoma （SC, 14 males and 2 females） , with a median age of 57 years （range, 46 to 70）. The survival analysis revealed that median survival time and the 1-, 3-, 5-year survival rates for these 3 groups were 20 months, 61.8%, 29.4%, and 20.6% in the CCC patients, 13 months, 52.6%, 51.6%, and 0% in the GCC patients, and 8 months, 31.3%, 0%, 0% in the SC patients, respectively. The median survival time and survival rate of the SC group were significantly lower than those of the other three groups （ P 〈 0.05 ）. However, in the SC group, the incidences of hilar lymph nodes metastasis, vascular tumor emboli and invasion of adjacent organs were significantly higher than those in the other three groups （P 〈 0.05 ）. There were no statistically significant differences among the other three groups （ P 〉 0.05 ）. The levels of carcino-embryonic antigen were higher in the three rare subtype groups than that of the control group. The incidences of multiple tumors of the three rare subtype groups were higher than that of the control group （P 〈 0. 05 ）. Positive surgical margin was an independent unfavorable prognostic factor. Conclusions The combined hepatocellular carcinoma and cholangiocarcinoma, giant cell carcinoma and sarcomatoid carcinoma have a poor prognosis. Among them sarcomatoid carcinoma is the most malignant and poor prognostic one. Radical resection is recommended.