中文摘要：

目的：通过研究肝脏少见原发恶性肿瘤临床病理的特点,以提高该类型肿瘤的诊疗水平。方法：收集我院肝胆外科自2003年1月至2012年12月经病理确诊的肝脏少见原发恶性肿瘤24例,其中经外科手术治疗20例,穿刺活检4例,结合文献对其临床及病理特点进行分析总结。结果：此组共24例,占同期全部肝脏恶性肿瘤病例的2.75%（24/874）,涵盖13种病理类型：肝母细胞瘤5例,神经内分泌瘤4例,淋巴瘤3例,胆管黏液腺癌2例,恶性神经鞘瘤2例,恶性神经束膜瘤1例,上皮样血管内皮瘤1例,上皮样血管肌肉脂肪瘤1例,多形肉瘤1例,癌肉瘤1例,平滑肌肉瘤1例,梭形细胞瘤1例,鳞癌1例。男女比例2∶1（16∶8）。平均年龄48.90岁（6~74岁,中位年龄51岁）。该组患者术后1年和5年生存率分别为49.0%和26.1%。结论：肝脏少见原发性恶性肿瘤临床诊断和鉴别困难,手术切除或穿刺活检的标本行免疫病理检查是诊断疾病的金标准。治疗尽量根治切除并按照指南辅助有效化疗或进行靶向治疗。

英文摘要：

Objective：To investigate the clinicopathologic characteristics of rare primary liver malignant tumor, im- proving the accuracy of diagnosis and treatment level. Methods：24 cases of rare primary liver malignant tumor （20 cases underwent surgical resection,4 cases underwent biopsy） which were confirmed by histopathology and immuno- histoehemistry,were collected in our hospital from Jan. 2003 to Dec. 2012. A retrospective analysis was made on these cases combined with literature. Results：Rare primary malignant liver tumor accounted for 2.75% （24/874） of all ca- ses of primary liver tumor over the same period, including 13 kinds of pathology ： Hepatoblastoma （ n = 5 ）, neuroendo- crine neoplasms （ n = 4）, lymphoma （ n = 3 ）, cholangiocarcinoma （ n = 2）, malignant neurilemmoma （ n = 2）, malig- nant perineurioma （ n = 1 ）, epitheliod hemangioendothelioma （ n = 1 ）, mucinous epithelioid angiomyolipoma （ n = 1 ）,pleomorphic sarcoma （ n = 1 ）, carcinosarcoma （ n = 1 ）, leiomyosarcoma （ n = 1 ）, spindle cell carcinoma （ n -- 1 ）, squamous cell carcinoma （ n = 1 ）. In this group, the ratio of male to female was 2：1 （ 16： 8）, with a mean age of 48.90 years old （6 ~74 years old,a median age of S1 years old）. The 1 and 5 years postoperative survival rate were 49.0% and 26.1%. Conclusion：It is difficult to make a clinical diagnosis on rare primary malignant liver tumor. Specimens from surgical resection or biopsy for immune pathology examination are the gold standard for diagnosis. Radical resec- tion and effective chemotherapy or tar~etin~ theraDv accordin~ to ~uidalin,= ~r,~ n,~,. ＋~＋~＋