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目的 探讨肺上皮样血管内皮瘤的临床病理特点。方法 4例肺上皮样血管内皮瘤, 3例女性, 1例男性,年龄 28 ~40岁,无自觉症状或有轻度咳嗽、气短。肺活检或手术切除标本经甲醛固定,石蜡包埋,常规HE及免疫组织化学 (Envision法 )染色。所用抗体包括CD31、CD34、细胞角蛋白(AE1 /AE3)、TTF -1、波形蛋白和上皮膜抗原。结果 本组肺上皮样血管内皮瘤病例女性多于男性,胸部CT显示双肺多发弥漫性小结节影。病理形态特点为结节周边上皮样肿瘤细胞呈花冠状充填于肺泡腔,病变中心为黏液透明样变间质,肺泡壁结构保留,肿瘤细胞胞质内有空泡形成,空泡内偶见红细胞,肿瘤细胞异型性不明显,核分裂和坏死均少见,免疫组织化学染色示CD31、CD34阳性,AE1 /AE3偶见灶状阳性,其他抗体呈阴性。结论 肺上皮样血管内皮瘤是一种具有独特临床病理特点的低度恶性血管来源肿瘤。
Objective To investigate the clinicopathological features of pulmonary epithelioid hemangioendothelioma. Methods 4 cases of pulmonary epithelioid hemangioendothelioma, 3 women, 1 male, aged 28 to 40 years old, no symptoms or mild cough, shortness of breath. Lung biopsy or surgical specimens were fixed with formaldehyde, embedded in paraffin, routine HE staining and immunohistochemistry (Envision method) staining. Antibodies used include CD31, CD34, cytokeratins (AE1 / AE3), TTF-1, vimentin and epithelial membrane antigens. Results This group of patients with pulmonary epithelioid hemangioendothelioma more than men, chest CT showed multiple pulmonary multiple diffuse nodules. Pathological features of the nodular pericyte-like tumor cells were coronal filling in the alveolar space, the center of the lesion was mucinous transparent interstitial, alveolar wall structure retained, the cytoplasm of tumor cells have vacuoles, occasional vacuoles within the red blood cells , Tumor cell atypia is not obvious, mitotic and necrotic are rare, immunohistochemical staining showed CD31, CD34 positive, AE1 / AE3 occasional focus-like lesions, other antibodies were negative. Conclusions Pulmonary epithelioid hemangioendothelioma is a low-grade malignant angiogenic tumor with unique clinicopathological features.