上皮样肉瘤样血管内皮细胞瘤临床病理观察

Clinicopathological features of epithelioid sarcoma-like hemangioendothelioma

目的分析上皮样肉瘤样血管内皮细胞瘤(ES-H)的临床病理学特点及诊断要点。方法通过HE切片、免疫组化观察1例上皮样肉瘤样血管内皮细胞瘤,并结合文献讨论。结果主要病理学形态表现为瘤细胞呈实性片状或巢状分布,瘤细胞圆形或略呈梭形、多角形,胞质嗜酸性,胞质内可见空泡或血管腔形成。免疫组化示CK、vimentin、FLI-1和CD31均(+),而CD34和FⅧ(-)。结论 ES-H是一种罕见的肿瘤,生物学行为属于低度恶性,具有复发及远处转移的潜能,诊断需要依赖免疫组化,治疗及预后尚待观察。

Purpose To investigate the clinicopathological features and to explore the major diagnostic points of epithelioid sarcoma-like hemangioendothelioma （ES-H）. Methods One case of ES-H was studied histopathologically and immunohistochemically, followed by review of related literature. Results The tumor cells were round or spindle in different areas, both with abundant eocinophilic cytoplasm, arranged in sheets, fascicles or irregular patterns. Immunohistochemical examination indicated that the tumor cells were diffusely positive for CK, vimentin, FLI-1, CD31 and negative for CD34 and FⅧ. Conclusion ES-H is a rare, low grade tumor with the potential recurrence and distant metastasis. Immunohistochemistry plays an important role in its diagnosis and differential diagnosis. Treatment and prognosis are still undetermined.