上皮样血管内皮瘤22例临床分析

Clinical analysis of epithelioid hemangioendothelioma

目的探讨上皮样血管内皮瘤(EHE)的临床病理学特点、鉴别诊断及预后。方法对22例手术切除或穿刺诊断的EHE临床和病理学特点进行分析,对免疫组化标志物的表达状况进行检测。结果22例EHE患者,年龄4～73岁,平均年龄44.2±1.3岁,发病以中年男性为主。肿瘤分别位于软组织、骨、肺、肝、脑,除肝脏外均为单个结节。17例行手术切除,1例肝动脉化疗栓塞治疗,1例CT引导下氩氦刀治疗,1例激光PDT治疗,1例对症治疗,1例放弃治疗。随访1个月～15年,16例患者健在,5例患者死亡,1例失访。其中5例术后40d～7年复发,4例发生局部侵犯,5例广泛转移。EHE组织学特征为具有血管分化的树突状细胞和有胞质内血管腔的上皮样细胞,间质致密纤维化及黏液样变。免疫组化分析表明,EHE至少表达CD31、CD34、FⅧRAg中的1项。结论EHE是一种低度恶性软组织肿瘤,其病因学尚不清楚。熟悉其临床及病理特征对避免漏诊及误诊具有重要意义。早期发现、局部手术扩大切除和结合术后放化疗或肝移植有助于延长患者生存期。

Objective To analyze clinicopathological features,differential diagnosis and prognosis of epithelioid hemangioendothelioma（EHE）by analysing clinical datas from January 1993 to December 2008. Methods The clinical and pathological features of 22 paitents of EHE were systematically observed and the antibodies were detected with immunohistochemistry. Results EHE predominantly occurred in middle-aged men with age ranging from 4-73 （mean 44.2）. The ratio of male to female was 1.4：1. The tumors located in soft tissue,bone ,lung,liver, and brain. 20 cases （90%）were with mononodular EHE except for 2 cases（10%）. Seventeen patients of 22 cases were surgically resected. Follow-up of 21 cases found that 4 cases developed local recurrences and 5 cases of them metastasized. Sixteen patients were still alive in 1 month-15 years after systematical therapy. Two of five patients metastasized were still alive in 2 months- 38 months. The other 3 died within 4-23 months. Histologically,the tumor consisted of dendritic and epithelioid cells that frequently contained vacuoles representing intracellular vascular lumina. The stroma was densely distributed with fibers and had myxohyaline areas. Immunohistochemically, all tumors were positive for CD31,CD34 and factor V$, whereas Vimentin and cytokeratin was occasionally expressed. Conclusions EHE is a distinctive low-grade sarcoma whose etiology remains unknown. Recognizing its clinical and morphological characters is important to avoid confusion with other lesions. Enlarged surgical resection of isolated lesions is the main treatment of choice with unpredictable results reported for chemotherapy,radiotherapy,and resection of multiple lesions. Orthotopic liver transplantation may be considered as a potentially curative treatment for this rare form of tumour.