上皮样血管内皮瘤的临床病理分析

目的：探讨上皮样（组织细胞样）血管内皮瘤的临床病理学特点及其意义。方法：对9例上皮样血管内皮瘤进行光镜和免疫组织化学SP法检测,1例作电镜观察,结果：年龄16－47岁,平均32岁,男女性别差异无显著性意义。部位：头面部4例,上肢3例,下肢2例,形态特征：瘤细胞具有上皮样或组织细胞样的形态;瘤细胞圆形或多角开,三五成群呈小巢状,索状,不规则状排列,分布于粘液间质中;间质可显著或少量粘液样变或玻璃样变,瘤细胞内含有原始血管腔,核分裂角,多形性及坏死少见,部分病例伴有梭形细胞血管内皮瘤改变,1例见破骨细胞样的多核巨细胞,免疫组织化学检测7例,7例波形蛋白均阳性,5例第八因子相关抗原,CD31,CD34阳性,2例细胞角蛋白弱阳性,1例CD68和a1-抗胰蛋白酶阳性,电镜观察1例见胞质内含丰富的微丝和少许的W－P小体,6例随访－8年,3例在原发部位有1或2次复发,但未见有转移,结论：上皮样血管内皮瘤是一种低度恶性的软组织肉瘤,其病因学尚不清楚,熟悉其形态特征对避免误诊为其他类似病变具具有重要意义。

A clinicopathologic study on 9 cases of epithelioid hemangioendothelioma

OBJECTIVE: To study the clinicopathological features of epithelioid (histiocytoid) hemangioendothelioma (EHE). METHODS: 9 cases of EHE were examined by light microscopy and immunohistochemistry, 1 case with ultrastructural observation. RESULTS: The patients' age ranged from 16 to 47 years (mean 32 years). The lesions occurred in both sexes and presented at a variety of sites including head and facial area (4 cases), upper limbs (3 cases) and lower limbs (2 cases). The morphological characters were: (1) "epithelioid" or "histiocytoid" tumor cells; (2) rounded or polygonal tumor cells arranged in nests, cords and irregular patterns; (3) the tumor cells were embedded in myxoid or hyaline matrix; (4) primitive lumen formation within a single tumor cell (intracytoplasmic lumens); (5) lack of pleomorphism, mitotic activity and necrosis; (6) presence of spindle cell hemangioendothelioma in some cases; (7) osteoclast like giant cells scattered throughout the entire lesion were observed in one case. Immunohistochemistry studies on 7 cases found that tumor cells were positive for vimentin (7/7), factor VIII-RAg (5/7), CD31 (5/7), CD34 (5/7), cytokeratin (2/7), CD68 and alpha-AT (1/7). Ultrastructural study of 1 case demonstrated Weibel-Palade bodies and an abundance of intermediate filaments in the cytoplasm. The follow-up of 6 cases found that 3 cases developed local recurrences but none of them metastasized. CONCLUSIONS: EHE is a distinctive low-grade sarcoma whose etiology remains unknown. Recognizing its morphological characters is important to avoid confusion with other similar lesions.