Zhu J X, Xie Q, Zhong A H, Lin Q H, Lan C Q
Department of Oncology, Fuzhou Pulmonary Hospital, Fuzhou 350008, China.
Pathology Department, Fuzhou Pulmonary Hospital, Fuzhou 350008, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2021 Nov 12;44(11):966-971. doi: 10.3760/cma.j.cn112147-20201218-01174.
To improve the diagnosis and treatment of pulmonary epithelioid hemangioendothelioma (P-EHE). Sixteen patients diagnosed with P-EHE in Fuzhou Pulmonary Hospital of Fujian Province from January 2009 to July 2020 were collected. Their gender, age, imaging findings, pathological characteristics, treatment protocols, survival and other clinical data were summarized and analyzed. The ratio of male to female among the 16 patients was 1∶1; and the average age of onset was 47.75 years. Most cases of PEHE (9/16) were found by physical examination, while some cases developed respiratory symptoms such as cough, sputum, shortness of breath, hemoptysis, chest pain, . (7/16). In most patients the lesions were localized to the lungs (11/16), while bone metastasis (1/16), lymph node metastasis (1/16), and pleural metastasis (4/16) also occurred. The pathological tissues were obtained mainly through surgical thoracoscopy. Chest CT images showed multiple nodules in both lungs, with most of the nodules less than 2.0 cm in diameter, and calcifications were seen, while solitary nodules and masses were rare, and pleural metastases could be manifested as pleural thickening and pleural effusion. The pathological findings were well-defined eosinophilic nodules with irregularly arranged nest-like structures. Those eosinophilic nodules had few central cells and abundant peripheral cells, which extended into the alveolar cavity like papillae. The tumor cells were epithelioid with small atypia, and vacuoles and red blood cells could be seen in the cytoplasm of individual tumor cells. Immunohistochemically, the tumor cells were positive to CD34, CD31, Factor Ⅷ andvimentin (VIM). Follow-up of 0.5 to 11 years showed that four patients died, two lost to follow-up, and the rest of the patients were in good condition, with a median overall survival (OS) of 4.58 years. PEHE is a rare low-grade lung tumor with no specific clinical manifestations. It can be diagnosed with chest imaging and pathological immunohistochemistry. Moreover, there is currently no standard treatment for PEHE, and most patients have a good prognosis.
为提高肺上皮样血管内皮瘤（P-EHE）的诊断与治疗水平。收集了2009年1月至2020年7月在福建省福州肺科医院确诊为P-EHE的16例患者。对其性别、年龄、影像学表现、病理特征、治疗方案、生存情况等临床资料进行总结分析。16例患者中男女比例为1∶1；平均发病年龄为47.75岁。多数PEHE病例（9/16）通过体格检查发现，部分病例出现咳嗽、咳痰、气短、咯血、胸痛等呼吸道症状（7/16）。多数患者病变局限于肺部（11/16），同时也出现骨转移（1/16）、淋巴结转移（1/16）和胸膜转移（4/16）。病理组织主要通过手术胸腔镜获取。胸部CT图像显示双肺多发结节，多数结节直径小于2.0 cm，可见钙化，孤立结节及肿块少见，胸膜转移可表现为胸膜增厚和胸腔积液。病理表现为边界清晰的嗜酸性结节，呈不规则排列的巢状结构。这些嗜酸性结节中央细胞少，周边细胞丰富，呈乳头样延伸至肺泡腔。肿瘤细胞呈上皮样，异型性小，个别肿瘤细胞胞质内可见空泡和红细胞。免疫组化显示肿瘤细胞CD34、CD31、因子Ⅷ和波形蛋白（VIM）呈阳性。0.5至11年的随访显示，4例患者死亡，2例失访，其余患者情况良好，中位总生存期（OS）为4.58年。P-EHE是一种罕见的低级别肺部肿瘤，无特异性临床表现。可通过胸部影像学及病理免疫组化进行诊断。此外，目前P-EHE尚无标准治疗方案，多数患者预后良好。
