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目的探讨肺胸区上皮样血管内皮瘤(epithelioid hemangioendothelioma,EHE)的临床病理学特点、治疗及预后。方法回顾性分析2例EHE的临床病理学资料,并复习相关文献。结果男性和女性患者各1例,年龄分别为21岁和52岁,影像学示左侧胸膜增厚、乳腺包块或左上肺结节影。组织学特征性地出现异型的嗜酸性上皮样瘤细胞、空泡状假脂肪母细胞,含单个红细胞的原始血管腔。免疫组化至少1项血管内皮标记阳性。例1瘤细胞中度异型,见瘤巨细胞,核分裂象多,组织结构多样,伴有肿瘤坏死,未行放、化疗,短期内死亡。例2纤维支气管镜活检示瘤细胞轻度异型,核分裂少,未见坏死,行放疗及2个疗程化疗,带瘤生存。结论 EHE根据其临床病理学特点,结合免疫表型可确诊。需与结核病、上皮样血管肉瘤、腺癌、恶性间皮瘤、黑色素瘤等鉴别。单发的经典型EHE者预后较好,而核分裂象多与肿瘤直径>3 cm的恶性EHE、胸膜起源、多中心或多脏器发生者预后差。
Objective To investigate the clinicopathological features, treatment and prognosis of epithelial hemangioendothelioma (EHE). Methods The clinical pathology data of 2 cases of EHE were retrospectively reviewed and the related literatures were reviewed. Results One male patient and one female patient were aged 21 years and 52 years respectively. Imaging showed left pleural thickening, breast mass or left upper pulmonary nodule. Histologically, abnormally shaped eosinoid epithelioid tumor cells, vacuolar pseudofollicular cells, and primitive blood vessel chambers containing a single erythrocyte were found. Immunohistochemistry of at least 1 positive endodermal marker. Example 1 tumor cells moderately shaped, see giant tumor cells, mitotic as many organizational structures, accompanied by tumor necrosis, did not put radiotherapy, chemotherapy, short-term death. Example 2 fiberoptic bronchoscopy biopsy showed mild dysmorphic tumor cells, less mitotic, no necrosis, radiotherapy and 2 courses of chemotherapy, tumor-bearing survival. Conclusion According to its clinicopathological characteristics, EHE can be diagnosed with immunophenotype. Need with tuberculosis, epithelioid angiosarcoma, adenocarcinoma, malignant mesothelioma, melanoma and other identification. A single classic EHE has a better prognosis, whereas a mycosis is more likely to be associated with a malignant EHE with a tumor diameter> 3 cm, a pleural origin, and a multicenter or multiple organ with poor prognosis.