儿童不消退型先天性血管瘤的临床病理特征及鉴别诊断

Clinicopathologic features and differential diagnoses of non-involuting congenital hemangioma in children

目的探讨不消退型先天性血管瘤（NICH）患儿临床病理特征及鉴别诊断。方法对22例NICH临床资料、组织形态和免疫组织化学进行分析。结果22例NICH患儿男14例，女8例，就诊平均年龄4．2岁。发病部位：头面部5例、颈部3例、躯体6例、上肢5例、下肢3例。组织学呈不规则的结节状或小叶状结构，血管内皮细胞核突出血管腔内，呈现“钉突样”，结节中央通常可见1条或多条管壁厚薄不一的引流血管；小叶间为纤维组织间隔和纤维脂肪间隔，纤维组织中通常可见异常的动脉和静脉结构，小叶边缘可见淋巴管样的结构；3例可见血栓。免疫组织化学均表达CD34（22／22）、CD31（22／22）、平滑肌肌动蛋白（22／22）、波形蛋白（22／22），D2-40表达位于结节边缘，所有病例不表达Glutl。结论NICH属于良性病变，预后较好，病理诊断需结合临床表现和组织形态特点，免疫组织化学有助于诊断和鉴别诊断。

Objective To investigate the clinicopathologic features and differential diagnoses of non-involuting congenital hemangioma （NICH） in children. Methods The clinical, morphologic and immunophenotypic characteristics of 22 cases of NICH were retrospectively analyzed. Results The mean patients＇ age at diagnosis was 4. 2 years, with a male to female ratio of 1.75： 1. The tumors were located in the head and face （5 cases）, neck （3 cases）, body （6 cases）, upper limbs （5 cases）, and lower limbs （ 3 cases）. Histologically, the tumor was dominated by rather large lobules of small vessels that were mostly rounded, curved, small and thin-walled, and were lined by endothelial ceils surrounded by one or more layers of pericytes. The center of the lobules was occupied by one or more thin or thick walled vessels, which were surrounded by fibrous and fatty tissue, which contained abnormal arterial and venous structures. At the edge of the lobules there were lymphatic vessels. Immunohsitochemical study showed that tumor cells in NICH were positive for CD34 （ 22/22 ）, CD31 （ 22/22 ）, SMA （ 22/22 ）, vimentin （ 22/22 ） and Glutl （0/22）. D2-40 expression was located at the edge of the capillary lobules. Conclusions NICH is a benign lesion. Clinically and pathologically, it needs to be differentiated from rapidly involuting congenital hemangioma, infantile hemangiomas, tufted angioma, vascular malformation, and others.