骨上皮样血管内皮瘤3例临床病理分析

目的 探讨骨上皮样血管内皮瘤 (epitheliod hemangioendothelialoma, EHE)的临床病理特征、诊断及鉴别诊断。方法 观察了3例骨的EHE形态学及免疫组织化学染色特点并复习相关文献。结果3例患者中，女性2例、男性1例。年龄14、35、36岁，平均28.3岁。病变均发生于下肢且呈多灶性。最常见的临床表现为局部疼痛。影像学显示边界清楚或模糊的溶骨性病变伴有硬化边。大体显示肿瘤呈卵圆形，切面灰褐色，质韧，出血明显。形态学上，肿瘤由大的多边形上皮样细胞及梭形细胞组成。两种细胞具有嗜酸性胞质、圆形或拉长的核及显著的核仁，分布于玻璃样变或黏液样基质中。在高分化区域，肿瘤细胞排列成条索或巢状伴随狭窄的小管或裂隙。但在分化差的区域，一些细胞呈弥漫实性或片层状，缺乏血管源性肿瘤的组织学表现。一些细胞出现代表原始血管的胞质内空泡，其内包含红细胞或碎片。免疫组织化学检查肿瘤细胞同时表达CD31、CD34(或FⅧRAg)及CK。辅以放、化疗的扩大切除术是治疗骨EHE的首选方法。结论 骨的上皮样血管内皮瘤是一种罕见的恶性血管源性肿瘤，需与转移癌、骨上皮样血管瘤和上皮样血管肉瘤等鉴别。

Clinicopathological Analysis of 3 Cases of Osseous Epitheliod Hemangioendothelialoma

Objective To investigate the clinicopathologic characteristics, diagnosis and differential diagnosisof osseous epitheliod hemangioendothelialoma (EHE). Methods Three cases of osseous EHE were analyzedon morphological and immunohistochemical staining features with review of the related literature. ResultsThe age of 3 patients (2 female and 1 male) was from 14 to 36 years (mean 28.3 years). All lesions located in thelower limbs and were multifocal. The most common symptom of EHE was localized pain. Imaging examinationrevealed osteolytic lesion with well- or poorly-demarcated margins and variable peripheral sclerosis. Grossly,the tumors were ovoid, rubbery and brow or tan with haemorrhage. Microscopically, the tumor was composedof large polyhedral epithelioid and spindle-shaped cells. Embedded within a hyaline or myxoid matrix, both hadabundant eosinophilic cytoplasms, round or elongated nuclei and prominent nucleoli. In well-differentiationparts, the tumor cells were arranged in cords or nests patterns accompanied with narrow channels or clifts.Some cells were in a diffused solid or sheet style and lack of histological profile of vascular tumor in illdifferentiationareas. And some cells contained intracytoplasmic vacuoles which represented primitive vascularlumina and they may harbor intact or fragments of red blood cells. Immunohistochemically, CD31,CD34 (or FⅧRAg) and CK were expressed in the tumor cells. Wide resection of the tumor was the preferred choice forthe treatment for EHE, combined with adjuvant radiotherapy or / and chemotherapy. Conclusion OsseousEHE is a rare type of vascular tumor with malignant behavior, and the differential diagnosis includes metastasiscarcinoma, epitheliod haemangioma and epitheliod angiosarcoma, etc.