肺上皮样血管内皮瘤临床病理特点分析

Clinicopathological charateristics of pulmonary epithelioid haemangioendothelioma

目的 分析肺上皮样血管内皮瘤(pulmonary epithelioid haemangioendothelioma,PEHE)的临床及病理特点、免疫组化染色结果、治疗方法及预后。方法 回顾性分析国家癌症中心/中国医学科学院肿瘤医院2011—2019年经外科手术切除及穿刺活检病理确诊的PEHE 10例,汇总整理患者性别、年龄、临床症状、肿瘤大体表现、镜下组织学特征、免疫组织化学染色结果、治疗及随访情况等临床病理信息。结果 10例PEHE中,男性2例,女性8例。发病年龄29~69岁,平均年龄48.6岁。2例呈转移性PEHE,8例呈原发性PEHE。出血(3例,30%)及疼痛(2例,20%)为最常见的临床症状。PEHE大体检查呈显著多灶发生(9例,90%),显微镜下呈多结节状生长方式,肿瘤细胞分布疏密不均,细胞界限不清,呈条索状、簇状、腺管样及单个细胞排列,可见含有单个红细胞的原始血管腔形成。2例手术病例出现局部淋巴结转移(2/8)。免疫组化染色呈显著的血管内皮标记物阳性特点,CD31(10/10)、CD34(10/10)、第Ⅷ因子相关抗原[F8,(7/8)]、Fli1(5/5)、ERG(1/1)及D2-40(1/1)。Ki-67增殖指数8例(8/10)小于10%,总体呈低增殖活性的表达状态。8例外科手术切除患者中3例随访期间死亡。结论 PEHE是罕见的血管源性肿瘤,穿刺诊断困难,需行免疫组化检测明确诊断。PEHE以多灶发生为主,可伴肝转移,伴有胸膜侵犯及淋巴结转移的病例预后不良,建议采取手术为主的综合治疗手段,并长期随访改善预后。

Objective To investigate the clinicopathologic characteristics,immunohistochemical result,treatment and prognosis of pulmonary epithelioid haemangioendothelioma(PEHE).Methods The clinical and pathological information of 10 PEHE patients was reviewed and analyzed in Cancer Hospital,Chinese Academy of Medical Sciences from 2011 to 2019,including gender,age distribution,clinical symptoms,gross and microscopic morphological features,immunohistochemical result,treatment and prognosis.There were 2 males and 8 females,with a median age of 48.6 years(range 29-69 years),including 2 cases of metastatic PEHE and 8 cases of primary PEHE.Hemoptysis(3 cases,30%) and pain(2 cases,20%) were the most common clinical symptoms.PEHE had a multifocal growing pattern in most(9 cases,90%) of our cases.Under the microscope,the tumor formed nodular structures,with various shapes and uneven distribution.The dense areas of tumor cells were arranged as cords,clusters,glandular tubes and single cells.Signet-ring like cells were present as well.Vacuoles and nuclear deviation were found in the cytoplasm,containing primitive vascular lumen filled with a single red blood cell.Local lymph node metastasis occurred in 2 post-surgery cases(2/8).Immunohistochemical staining result showed significant positive signals of vascular endothelial markers,including CD31(10/10),CD34(10/10),F8(7/8),Fli1(5/5),ERG(1/1) and D2-40(1/1).Ki67 proliferation index was less than 10% in 8 cases(8/10),with a predominantly low proliferative lesion.Among the 8 post-surgery patients,3 died during the follow-up period.Conclusion Pulmonary epithelioid hemangioendothelioma(PEHE) is a rare vascular tumor,which is difficult to diagnose in biopsy.Immunohistochemical staining is necessary for the correct diagnosis.It always occurs in multiple foci,and the hepatic metastasis could be the comorbidity.Patients with pleura invasion and lymph node metastasis are related to worse prognosis.It is recommended to take comprehensive treatment based on surgery,and insist on long-term follow-up to improve the prognosis.