先天性血管瘤19例临床病理分析

Clinicopathological analysis of 19 cases with congenital nonprogressive hemangiomas

目的探讨小儿先天性血管瘤(congenital hemangiomas,CH)的临床病理学特征、诊断及鉴别诊断。方法收集19例CH的临床、病理资料及随访结果,应用HE染色及免疫组化EnVision两步法观察组织病理学特征。结果 19例患者中男性13例,女性6例,平均年龄52.4个月,中位年龄48个月。9例位于四肢及躯干,10例位于头面部;其中1例为多发病灶。病理大体标本为0.4~7 cm的皮肤及皮下组织,皮肤表面可见形状不规则的灰红、暗红色实性肿块,表面平坦或局部隆起,部分病例中央出血及坏死。镜下肿瘤组织由增生血管内皮细胞和血管周细胞组成的毛细血管排列成小叶状。部分患者病变中央有退化区域。小叶之间为丰富的纤维组织,部分有胶原变性,可见较粗大的引流血管。19例肿瘤细胞均表达CD31、CD34和WT1;GLUT1、D2-40均阴性。所有病例手术切除治疗,随访时间1~24个月,均无复发及转移。结论 CH属于少见的小儿先天性良性血管肿瘤,临床病理特及组织学形态均较为特殊,对该少见类型的血管瘤明确其诊断及鉴别诊断,对临床治疗有很重要意义。

Purpose To investigate the clinicopathological features and differential diagnosis of congenital nonprogressive hemangiomas in children. Methods The clinic pathologic data and follow-up results of 19 cases of congenital nonprogressive heman- giomas were collected. The histopathologie features of congenital nonprogressive hamangiomas were observed with HE staining and immunohistochemistry of EnVision two-step. Results Of the 19 patients, 13 were males and 6 were females, with an average age of 52.4 months and medium age of 48 months. 9 cases occurred in the limbs and trunk, 10 lesions were located in head and face included 1 case with muhifocal lesions. Grossly, the specimens were irregular solid mass with range from 0.4 cm to 7 cm in maxi- mum diameter. Microscopically, the lesions were composed of capillary lobules that separated by abnormal dense fibrotic dermal stroma typically containing a prominent component of large vessels. Immunohistochemically, the tumor cells were positive for CD31, CD34, and WT1, but negative for D2-40 and GLUT1. All cases underwent surgical resection, and the follow-up period was 1-24 months. There was no recurrence or metastasis. Conclusion Congenital nonprogressive hemangioma is a rare benign vascular tumor in children, with characteristic clinical and pathologic features. So the exact diagnosis is very important for both patholo-gists and clinicians.