上皮样血管内皮瘤的临床病理分析

A clinicopathologic study on 9 cases of epithelioid hemangioendothelioma

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 ;瘤细胞圆形或多角形 ,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 ;核分裂象、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CD6 8和α1 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 2～ 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。

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 α-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.