肝脏少见原发性间叶性肿瘤101例的临床病理研究

目的：研究肝脏少见原发性良、恶性间叶性肿瘤的临床病理特点以提高病理诊断的准确性。方法：回顾性分析于1982年1月至2002年12月间经手术切除、组织病理和免疫病理诊断证实的原发性间叶性肿瘤共性18种101例（不包括肝海绵状血管瘤）。结果：肝良性间叶性肿瘤有7种48例，占47.5%(48/101)，男女之比为1：1.8，平均31.2岁；以肝血管平滑肌脂肪瘤最为常见，占70.8%(34/48)；该组患者术后至今全部生存，无1例复发。肝恶性间叶性肿瘤有11种53例，占52.5%（53/101），男女之比为1.2:1，平均44.1岁；其中最常见者为肝淋巴瘤20.8%(11/53)]，全部见于男性，且63.6%(7/11)的患者有HBV感染史，2例合并肝细胞癌；该组患者术后2年和3年生存率分别为61.9%(13/21）和33.3%(7/21)。结论：肝脏可以原发大部分类型的软组织肿瘤，良、恶性肿瘤患者之间的性别和年龄分布有所不同，值得在诊断时参考。在做出病理诊断之前必须结合临床排除转移；鉴别诊断应首先排队肝细胞癌与肝内胆管癌的梭形细胞型或局部内瘤样化等变异类型；科学、合理地使用免疫组化染色谱具有重要的诊断和鉴别诊断价值。

Clinicopathological study on 101 cases of rare primary liver mesenchymal tumors

Objective: To investigate the clinicopathologic characteristics of rare primary hepatic benign and malignant mesenchymal tumors,improving the accuracy of pathological diagnosis. Methods: A retrospective analysis was made on 101 cases of 18 kinds of hepatic mesenchymal tumors (not including cavernous hemangioma) , which were surgically resected in our hospital from Jan. 1982 to Dec. 2002 and were confirmed by histopathology and immunohistochemistry. Results: Hepatic benign mesenchymal tumors accounted for 47. 5%,including 48 cases of 7 kinds. In this group,the ratio of male to female was 1 : 1. 8,with a mean age of 31. 2 years old. The most common tumor was hepatic angiomyolipoma,which occurred in 34 cases (70. 8%). All of the patients in this group were alive without recurrence. Hepatic malignant mesenchymal tumors accounted for 52. 5% .including 53 cases of 11 kinds. In this group,the ratio of male to female was 1. 2: 1.with a mean age of 44. 1 years old. The 2 and 3 years survival rate was 61. 9% (13/21) and 33. 3% (7/21) respectively. The most common tumor was hepatic lymphoma,all of which occurred in males (n= 11, 20. 8%),and 63. 6%(7/11) of them was positive of serum HBsAg,and 2 were associated with hepatocellular carcinoma. Conclusion: Most kinds of soft tissue tumors can primarily occur in the liver. It is noteworthy that difference of age and sex distribution between patients with benign and malignant tumors provides information for diagnosis. Extrahepatic metastases should be firstly eliminated before final diagnosis. The differential diagnosis includes spindle-cell type or focal sarcomatous change of hepatocelluar carcinoma and intrahepatic cholangiocarcinoma. The reasonable use of immunohistochemcal staining has important diagnostic and differential diagnostic values.