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  • Title: [Often misdiagnosed primary pleural epithelioid hemangioendothelioma: a clinicopathological analysis of five cases].
    Author: Xie XF, Huang Y, Guo JH, Zhang W, Hou LK, Wu CY, Zhang LP.
    Journal: Zhonghua Bing Li Xue Za Zhi; 2020 Dec 08; 49(12):1288-1293. PubMed ID: 33287515.
    Abstract:
    Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of primary pleural epithelioid hemangioendothelioma (EHE). Methods: Five cases of pleural EHE diagnosed from 2012 to 2019 in the Shanghai Pulmonary Hospital of Tongji University, China, were collected. They were subjected to review of clinical and image data, reevaluation of histological sections, immunohistochemistry study, clinical follow up and literature review. Results: There were 1 male and 4 females (male to female ratio of 1∶4). The age ranged 19‒58 years, with a median age of 52 years. There was no smoking history in all 5 cases. Clinical manifestations included chest distress, chest pain, cough and hemoptysis. CT scan showed pleural effusion, pleural thickening, multiple pleural nodules and pneumothorax. All of the 5 cases were diagnosed based on thoracoscopic biopsies, which were performed for the suspicion of malignancy in 3 cases and for the suspicion of tuberculosis upon the failure of anti-tuberculosis treatment in 2 cases. Histologically, the tumors grew in the mesenchyme, forming cords, nests and solid sheets. The tumor cells were round, oval, short spindle, and polygonal in shape. They were epithelioid and had abundant cytoplasm and prominent nucleoli. Blister cells (vacuoles in cytoplasm) could be seen, and for mednewly-generated vessel lumen. Mucoid degeneration and collagenization were common in the tumor stroma. The positive-stainin grate was 5/5 for vimentin, 5/5 for D2-40, 4/5 for CD31, 4/5 for ERG, 4/5 for CAMTA1, 2/5 for CD34 and 2/5 for CKpan. WT1, calretinin and CK5/6 were all negative. The follow-up time ranged from 4‒18 months. During the follow-up, 4 patients were treated with chemotherapy (2 patients died, and 2 survived with tumor), and 1 patient lost to the follow-up. Conclusions: Primary pleural EHE is rare. Its imaging and clinical features are very similar to those of tuberculosis. The correct diagnosis relies on pathological biopsy. Tumor cells are epithelioid. When CD34 is negative and D2-40 is positive, it is easy to be misdiagnosed as malignant mesothelioma. The combination of CD31, ERG and CAMTA1, which have high specificity of EHE, is helpful for the differential diagnosis. 目的： 探讨原发于胸膜的上皮样血管内皮瘤（EHE）的临床病理学特征、诊断与鉴别诊断。 方法： 收集同济大学附属上海市肺科医院2012—2019年间诊断的5例胸膜EHE，查阅临床及影像学资料，复习组织学切片，补充免疫组织化学，随访并复习相关文献。 结果： 男性1例，女性4例，男女比例1∶4，年龄19~58岁，中位年龄52岁。5例均无吸烟史，临床表现为胸痛、胸闷、咳嗽、乏力、咯血，CT表现为胸腔积液、胸膜增厚、胸膜多发结节、气胸。5例均为胸腔镜手术活检样本，3例临床考虑恶性病变行活检，2例临床怀疑结核，行诊断性抗痨无效后活检。组织学上，肿瘤在纤维间质中呈条索样或者巢团状、实性片状浸润性生长，细胞呈圆形、卵圆形、短梭形、多角形，细胞上皮样，胞质丰富，有小核仁；可见水泡细胞（胞质内空泡），内含单个红细胞形成单内皮细胞血管腔。肿瘤间质常见黏液样变性、胶原化。免疫组织化学肿瘤细胞CD31阳性比例为4/5，ERG阳性为4/5，CD34阳性为2/5，广谱细胞角蛋白阳性为2/5，波形蛋白阳性为5/5，D2-40阳性为5/5，CAMTA1阳性为4/5。WT1、Calretinin、CK5/6均阴性。随访5例患者，随访时间4~18个月，4例行化疗，其中2例死亡、2例带瘤生存，1例失访。 结论： 胸膜原发EHE罕见，影像学与临床表现与结核性胸膜炎、恶性间皮瘤酷似，诊断依赖病理活检；肿瘤细胞呈上皮样，当CD34阴性、D2-40阳性时易误诊为恶性间皮瘤。联合应用EHE特异性高的标志物CD31、ERG、CAMTA1等有助于鉴别诊断。.
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