Yuan L, Wang F H, Li L P, Chen Z R, Chen K, Zeng L, Qin L J, Niu H L
Department of Pathology, Guangzhou Women and Children's Medical Center, Guangzhou 510623, China.
Zhonghua Bing Li Xue Za Zhi. 2021 Sep 8;50(9):1029-1033. doi: 10.3760/cma.j.cn112151-20201231-00993.
To investigate the clinicopathological features of hepatic vascular tumors in children. s 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. 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. 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.
探讨儿童肝血管肿瘤的临床病理特征。总结并分析了2007年9月至2020年11月在广州妇女儿童医疗中心收治的22例儿童肝血管肿瘤的临床特点、组织学及免疫组化染色结果。22例患者年龄从1.0个月至2.5岁（平均年龄9个月）。男10例，女12例。5例为早产儿且低体重；3例在孕期发现；1例同时患有皮肤血管瘤；6例伴有贫血；所有患者均未出现Kasabach-Merritt现象。CT检查显示17个肿瘤为单发，5个为多发病变。大体上，肿瘤大小为0.6 cm至11.0 cm；切面实性，呈灰红色和棕色，6例中央区域有出血和坏死。镜下，15例单发先天性肝血管瘤中央区域有特征性坏死，周边为疏松纤维组织。可见毛细血管增生、血管腔之间残留胆管以及扩张的血栓形成血管通道，包含髓外造血灶和钙化。5例多发婴儿型肝血管瘤由不同大小的毛细血管组成，内皮细胞和周细胞饱满，呈小叶状或弥漫性排列。2例海绵状血管瘤（静脉畸形）由扩张的薄壁血管组成，呈分支状，内衬扁平内皮细胞。免疫组化方面，22例均表达血管内皮标志物CD31和CD34，但D2-40为阴性。5例多发婴儿型肝血管瘤中Glut1阳性，其余病例为阴性。儿童肝血管肿瘤罕见，其分类与成人不同。明确病理诊断具有重要意义。
