41例肝脏原发性恶性血管肿瘤的临床病理特点

目的 探讨肝脏原发性恶性血管肿瘤（PHMVT）的临床病理学及预后的特点。方法 回顾性分析1982年1月至2012年12月在我院手术切除并经病理组织学证实的41例PHMVT，对其临床表现、病理学特点及预后进行分析。结果 41例患者中，肝上皮样血管内皮瘤（EHE） 19例（46.3％），平均瘤体直径为49cm（1.2～6.3cm）；肝血管肉瘤（PHA）14例（34.1％），平均瘤体直径为8.3cm（3.0～14.0cm）；肝婴儿型血管内皮瘤（IHE）5例（12.2％），平均瘤体直径为3.8cm（1.2～6.3cm）；肝恶性血管外皮瘤（MHP）3例（7.3％），平均瘤体直径为7.8cm（2.1～13.0cm）。免疫组化显示41例肿瘤细胞均表达Vimentin、CD34或因子Ⅷ。EHE患者术后中位生存期为87个月，优于PHA的12个月（P＜0.05）；2例有完整随访资料的MHP患者分别于术后43个月死亡和术后84个月复发；IHE患者术后均无复发，预后良好。结论 PHMVT的病理类型与预后有关，PHMVT的恶性程度由高到低依次为PHA＞MHP＞EHE＞IHE。

Clinicopathological features of primary hepatic malignant vascular tumors: A analysis on 41 cases

Objective To investigate the elinicopathological and prognostic features of primary hepatic malignant vascular tumor（PHMVT）. Methods Forty-one patients with PHMVT who underwent surgical resection and confirmed by pathology in our hos- pital from January 1982 to December 2012 were analyzed for their clinical manifestations, pathological characteristics and prognosis. Results Of the 41 patients, pathological classification contained 19 EHE（46. 3% ） ,14 PHA（34. 1% ） ,5 IHE（ 12. 2% ） and 3 MHP （7.3%）. The average diameters of the 4 kinds tumor were followed by 4. 9cm（ 1.2-6. 3 cm）, 8.3cm（3.0-14. 0 cm）, 3.8cm（ 1.2- 6. 3cm） and 7.8cm（2. 1-13era）. Immunohistochemistry showed all of 41 cases expressed Vimentin, CD34 or factor VⅢ. EHE patients showed better postoperative survival than PHA patients（87 months vs. 12 months, P 〈 0.05）. Two patients with MHP who had com- plete follow-up data, one patient relapsed at the 84th month postoperative, another died of disease in 43 months after operation. Pa- tients with IHE recovered well with no recurrence after operation. Conclusion The prognosis of patients with PHMVT is associated with pathological types ; and the malignant degree of PHMVT are in sequence of PHA 〉 MHP 〉 EHE 〉 IHE.