论文部分内容阅读
目的:分析肝脏原发的上皮样血管内皮细胞瘤(EHE)临床与病理表现,以提高对EHE的认识。方法:回顾性分析4例EHE的临床、影像、组织学形态及免疫组化表达及特殊染色结果,结合随访结果复习相关文献进行讨论。结果:4例患者中,男2例,女2例,年龄分别为55、36、41和44岁。临床表现为腹胀、右上腹痛,其中1例非常罕见的类似布加氏综合征(BCS)的表现。2例肝功能异常。影像学上均表现为肝内多发结节或弥漫性病变,临床考虑肝癌或BCS。3例为肝脏穿刺标本,1例为肝脏全切标本,后者切面见弥漫性灰白色病变,与周围肝组织分界不清。光镜下细胞形态以上皮样细胞为主,排列成短条索状或小巢状,包埋于特征性黏液玻璃样基质中。瘤细胞胞质丰富,可见明显的胞质内空泡似印戒样。3例穿刺标本细胞形态温和,未见核分裂相,2例有坏死。1例肝脏全切标本细胞异型性较明显,可见核分裂相,>1/10HPF,并见大片凝固性坏死。免疫组化示,CD31、CD34、F8及VEGF阳性,广谱CK偶见灶性阳性。特殊染色瘤细胞PAS及AB-PAS均阴性。随访2～22个月,1例肝移植术后死亡,2例出院后死亡,1例失访。结论:EHE为一种少见的恶性肿瘤,临床与影像学表现无特异性,极易误诊为其他肝脏肿瘤,需要引起重视。
Objective: To analyze the clinical and pathological features of primary hematogenous epithelial hemangioendothelioma (EHE) in liver so as to improve the understanding of EHE. Methods: Clinical, imaging, histological features, immunohistochemical staining and special staining of 4 cases of EHE were retrospectively analyzed. The related literatures were reviewed with the follow-up results. Results: Among the 4 patients, there were 2 males and 2 females with the ages of 55, 36, 41 and 44 respectively. Clinical manifestations of bloating, right upper quadrant pain, including 1 case of very rare manifestations of Budd-Chiari syndrome (BCS). 2 cases of liver dysfunction. Imaging showed multiple intrahepatic nodules or diffuse lesions, clinical consideration of liver cancer or BCS. 3 cases of liver puncture specimens, 1 case of liver resection specimens, the latter see diffuse gray lesions, and the surrounding liver tissue unclear boundaries. Under light microscopy, the epithelial cells were mainly epithelial cells arranged in short cords or small nests and embedded in the characteristic mucus-like matrix. Cytoplasm of tumor cells is rich, visible cytoplasmic vacuoles resemble signet samples. 3 cases of puncture specimens mild cell morphology, no mitotic phase, 2 cases of necrosis. One case of liver resection specimens of atypical cells more obvious, visible mitotic phase,> 1 / 10HPF, and see large coagulation necrosis. Immunohistochemistry showed that CD31, CD34, F8 and VEGF were positive, and the broad-spectrum CK was occasionally focally positive. Special staining of tumor cells PAS and AB-PAS were negative. One to two patients died after liver transplantation, two died after discharge and one lost to follow-up. Conclusion: EHE is a rare malignant tumor. Clinical and imaging findings are nonspecific and easily misdiagnosed as other liver tumors, which needs attention.