摘要目的:探讨儿童肝脏血管源性肿瘤的临床病理特征。方法:对2007年9月至2020年11月广州市妇女儿童医疗中心22例儿童肝脏血管源性肿瘤患者的临床特征、标本的组织学及免疫组织化学染色结果进行总结分析。结果:22例儿童肝脏血管源性肿瘤，年龄1个月至2.5岁，平均年龄9个月。男10例，女12例。早产儿及低出生体重儿5例，产前发现肝脏病变3例，伴有皮肤血管瘤1例，伴有贫血6例，均未出现Kasabach-Merritt现象。CT检查肝组织内孤立性病变17例，多灶性5例。病理学检查：肿瘤直径0.6~11.0 cm，切面实性，灰红灰褐色，6例中央见出血坏死。显微镜下，15例孤立性先天性肝血管瘤见特征性的中央坏死区，周围组织疏松，可见毛细血管增生，管腔之间较多残留小胆管，扩张的血管内血栓形成，其中可见髓外造血及钙化。5例多灶性肝婴儿血管瘤见毛细血管分叶状或弥漫排列，管腔大小不等，可见较肥胖的血管内皮细胞及血管周细胞。2例海绵状血管瘤（静脉畸形）由扩张的分支状薄壁血管组成，内衬扁平的内皮细胞。22例肝脏血管瘤均表达血管内皮标志物CD31及CD34，不表达D2-40。5例多灶性肝婴儿血管瘤Glut1阳性，其余Glut1均阴性。结论:儿童肝脏血管源性肿瘤罕见，其分型与成人不同，明确病理诊断具有重要意义。

abstractsObjective:To investigate the clinicopathological features of hepatic vascular tumors in children. Methods The clinical characteristics, histology and immunohistochemical staining results were summarized and analyzed in 22 cases of hepatic vascular tumors in children at Guangzhou Women and Children′s Medical Center from September 2007 to November 2020. Results:The 22 patients aged from 1.0 month to 2.5 years (mean age 9 months). There were 10 males and 12 females. Five cases were found in premature and had low birth weight infants; three cases were discovered in the antenatal period; one patient also had cutanous hemangioma; six patients had associated anemia; Kasabach-Merritt phenomenon was not seen in any patient. CT examination showed 17 tumors were solitary and five were multifocal lesions. Macroscopically, the tumors size ranged from was 0.6 cm to 11.0 cm; the cut surface was solid, gray red and brown in color, and in six cases there were hemorrhage and necrosis in the central area. Microscopically,15 cases of solitary congenital hepatic hemangiomas showed characteristic necrosis in the central area, with loose fibrous tissues at periphery. Proliferation of capillaries, residual bile ducts between the vascular lumens, and dilated thrombosed vascular channels were seen, and contained extramedullary hematopoietic foci and calcification. Five cases of multiple hepatic infantile hemangiomas showed capillaries of different sizes composing of plump endothelium and pericytes and were arranged in lobular or diffuse patterns. Two cases of cavernous hemangioma (venous malformation) consisted of dilated thin-walled blood vessels with branch-like pattern lined with flat endothelial cells. Immunohistochemically, all 22 case expressed vascular endothelial markers CD31 and CD34, but D2-40 was negative. Glut1 was positive in five cases of multiple hepatic infantile hemangiomas, and the other cases were negative.Conclusion:Hepatic vascular tumors in children are rare, and their classification is different from that of adults. It is of great significance to make clear pathologic diagnosis.