肺上皮样血管内皮瘤4例临床病理观察

Pulmonary epithelioid hemangioendothelioma:a clinicopathological analysis of four cases

目的探讨肺上皮样血管内皮瘤(PEHE)的临床病理学特征、免疫表型、鉴别诊断及预后。方法回顾性分析4例PEHE的临床病理学资料,对肿瘤标本行常规HE和免疫组化En Vision两步法检测,并复习相关文献。结果4例患者均为男性,平均年龄515岁,2例为肺内孤立性结节,2例为双肺多发小结节,其中1例双侧胸膜见多发结节。4例患者中,2例为体检发现肺结节,1例临床表现为咳嗽伴痰中带血,1例为左侧胸背部起疹伴疼痛于皮肤科行CT检查发现。镜下见肿瘤呈多结节状生长,中央区细胞稀疏、间质玻璃样变性或呈黏液软骨样,周边细胞密度增加,排列呈条索状、巢状或簇状,肿瘤细胞胞浆丰富呈上皮样或多边形,部分肿瘤细胞内可以观察到含有红细胞的原始血管腔形成。结节周围肿瘤细胞呈息肉状侵入肺泡腔。其中1例除上述结构外,局部出现肿瘤细胞密度增加、异型性明显等非典型形态。免疫表型:肿瘤细胞呈血管内皮标记物阳性,如CD31、Fli-1、CD34、ERG;4例均表达CAMTA1;2例上皮标记物PCK呈阳性表达;4例TFE3、EMA、TTF1、WT-1均呈阴性。本组4例患者中,1例术后经阿帕替尼靶向治疗,2例经紫杉醇+卡铂化疗并联合贝伐珠单抗治疗,随访9~69个月,现病情稳定。另1例术后于外院化疗,术后21个月死亡。结论PEHE是一种少见的肺间叶源性肿瘤,确诊主要依赖特征性的病理学形态和免疫表型,必要时行遗传学检查辅助诊断。

Objective To investigate the clinicopathological characteristics,immunophenotype,differential diagnosis,and prognosis of pulmonary epithelioid hemangioendothelioma(PEHE).Methods The clinicopathological data of 4 cases of PEHE were retrospectively analyzed.Tumor specimens were detected by routine HE and immunohistochemical Envision two-step methods,and the relevant literature was reviewed.Results The four patients were all male,with an average age of 515 years.Two patients had solitary pulmonary nodules,two patients had multiple small nodules in both lungs,and one patient had multiple nodules in both pleura.Of the 4 patients,2 were found to have pulmonary nodules during physical examination,1 had a clinical manifestation of cough with blood in sputum,and 1 had a rash on the left chest and back with pain,which was found on CT examination in the dermatology department.Microscopically,the tumor showed multinodular growth,with sparse cells in the central region,vitreous degeneration of the stroma,or mucilaginous cartilage like appearance.The density of peripheral cells increases,arranged in a strip,nest,or cluster shape.The cytoplasm of tumor cells is abundant,showing an epithelioid or polygonal appearance.In some tumor cells,the formation of primitive vascular cavities containing red blood cells can be observed.Tumor cells around the nodules invaded the alveolar space as polyps.In one case,in addition to the above structures,atypical morphology such as increased tumor cell density and marked atypia appeared locally.Tumor cells showed positive vascular endothelial markers,including CD31,Fli-1,CD34,and ERG.CAMTA1 was expressed in all 4 cases.Two cases showed positive expression of epithelial marker PCK.Four cases of TFE3,EMA,TTF1,and WT-1 were all negative.One of the four patients was treated with apatinib targeted therapy after surgery,and two patients were treated with paclitaxel plus carboplatin chemotherapy combined with bevacizumab.The patients were followed up for 9 to 69 months and were in stable condition.Another case underwent chemotherapy in an external hospital after surgery,and died 21 months after surgery.Conclusion PEHE is a rare mesenchymal tumor of the lung,and its diagnosis mainly depends on the characteristic pathological morphology and immunophenotype.If necessary,genetic examination should be performed to assist in the diagnosis.