肝上皮样血管内皮瘤6例

Six cases of hepatic epithelioid hemangioendothelioma

目的探讨肝上皮样血管内皮瘤(EHE)的临床及病理特点。方法观察6例肝EHE病理大体形态,并进行光学显微镜观察和免疫组织化学检测,随访患者的临床表现并复习文献。结果巨检肿瘤多见于肝右叶及肝被膜下,单发或多发,可累及左、右两侧肝叶,可见钙化及出血坏死;镜下瘤细胞由上皮样及树突样细胞组成簇状或条索状结构,胞质内血管形成,管腔可见红细胞、间质纤维化、玻璃样变及黏液样变性,瘤细胞向周围肝窦状隙及血管浸润性生长;文献报道较为特异性的免疫标记物FⅧRAg因子、CD31、CD34、Vimentin等有助于诊断。结论肝EHE是一种少见的低度恶性肿瘤,易误诊,了解该肿瘤的临床及病理特点有助于鉴别其他易混淆的肿瘤类型。

Objective To explore clinical and histopathological features of hepatic epithelioid hemangioendothelioma （EHE）. Methods Gross morphology and mieromorphology,immunohistochemistry and follow-up data of 6 EHEs were analyzed. Literature about EHE was reviewed. Results Most EHEs were solitary or multiple tumors located in the right lobe under the liver capsule or in both left and right lobes of the liver. Macroscopically, calcification, hemorrhage and necrosis could be found in the tumor; Histologically, the neoplastic cell exhibited epithelioid and dendritic features, arranged in clusters and streaks, or represented intracytoplasmic lumina containing erythrocytes, fibrosis, hyalinosis and myxoid degeneration. The neoplastic cell typically invaded the liver sinusoids and vessels. The immunostaining of F Ⅷ RAg cytokine, CD31, CD34, Vimentin were useful for the diagnosis of EHE. Conclusion EHE of the liver behaves as low-grade malignant neoplasm with low incidence. It can he misdiagnosed as carcinoma. Understanding the clinical and pathological features of EHE is helpful to differentiate it from other confusing diseases.