上皮样血管内皮瘤的诊断和治疗

Diagnosis and Treatment of Epithelioid Hemangioendothelioma

目的探讨上皮样血管内皮瘤的临床特点、诊断方法和治疗方式。方法回顾性分析2001年1月至2017年12月北京协和医院收治的30例上皮样血管内皮瘤患者的临床资料,对所有患者的辅助检查结果、治疗方法、病理结果和预后进行比较分析。结果 30例患者中,男10例,女20例,平均年龄(47. 9±16. 1)岁,肿瘤平均大小(5. 8±2. 8) cm。17例(56. 7%)在确诊前无不适主诉,17例(56. 7%)查体无阳性体征。最常见的发病部位是头皮和脊椎,其次是肝脏。19例患者接受手术治疗,11例患者仅行穿刺活检取病理。所有患者均病理诊断为上皮样血管内皮瘤。各项免疫组织化学指标中,CD34和CD31的阳性率最高。共23例患者获得随访,平均随访时间(74. 1±56. 8)个月。17例生存,6例死亡。自起病后1、3、5年的累积生存率分别为95. 7%、86. 3%、73. 6%。手术患者远处转移的比例(14. 3%比77. 8%,P=0. 007)和死亡率(7. 1%比55. 6%,P=0. 018)均明显低于活检患者。结论上皮样血管内皮瘤临床少见,无特异性症状体征,可发生于全身各个系统,诊断依赖病理证据,总体预后较好。手术完整切除病灶有助于降低远处转移率和死亡率。

Objective To evaluate the clinical feature,diagnosis,and treatment of epithelioid hemangioendothelioma(EHE).Methods Data of 30 EHE patients admitted from January 2001 to December 2017 were retrospectively analyzed.The auxiliary examinations,treatment method,pathological findings,and prognoses were analyzed.Results There were 10 male patients and 20 females aged(47.9±16.1)years.The lesions sized(5.8±2.8)cm.Seventeen cases(56.7%)were asymptomatic and 17(56.7%)had no positive sign.The most common locations of EHE were scalp and spine,followed by liver.Nineteen patients underwent surgical operation(surgery group)and 11 only accepted needle biopsy(biopsy group).EHE was pathologically confirmed in all cases.CD34 and CD31 showed the highest positive rates in immunohistochemical stains.Twenty-three cases were followed up by(74.1±56.8)months.Seventeen cases survived and 6 died.The 1-,3-,and 5-year cumulative survival rates were 95.7%,86.3%,and 73.6%,respectively.The metastasis(14.3%vs.77.8%,P=0.007)and mortality(7.1%vs.55.6%,P=0.018)rates were significantly lower in surgery group than in biopsy group.Conclusions EHE is a rare tumor without specific symptom or sign.It can occur in any system of the body.Diagnosis depends mainly on pathology.The prognosis is acceptable.Complete surgical resection reduces distant metastases and mortality.