婴儿型血管外皮细胞瘤9例临床病理分析  

Infantile hemangiopericytomas: a clinicopathologic analysis of nine cases

作　　者：周峥珍[1] 陈卫坚[1] 姜楠[1] 张丽琼[1] 刘敏[1] 

机构地区：[1]湖南省儿童医院儿科医学研究所,长沙410007

出　　处：《诊断病理学杂志》2015年第5期276-279,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的 探讨婴儿型血管外皮细胞瘤（HPC）的临床病理特征、诊断与鉴别诊断.方法 回顾性分析9例婴儿型血管外皮细胞瘤病例的临床病理学特征、免疫组化结果,并复习相关文献.结果 男婴6例,女婴3例,中位年龄1.6个月.主要症状为局部肿块.肿块平均直径4.6 cm.8例为良性,1例为低度恶性;随访7例,2～ 36个月,无复发及转移.HPC由丰富血管网及周围紧密排列的圆形或梭形瘤细胞组成,血管扩张,大小不一,可呈特征性“鹿角状”薄壁分支血管,细胞密度大,核分裂象多,并可见坏死及钙化.免疫组化：瘤细胞vimentin均（＋）,部分病例CD34、CD31、Ⅷ因子、CD99、bcl-2和actin（＋）,Ki-67增殖指数5％ ～ 30％,平均12％.结论 婴儿型血管外皮细胞瘤属少见软组织肿瘤,临床及影像学无特征性,组织学形态结合免疫组化是鉴别诊断的最可靠方法.Objective To investigate the clinical pathological features, pathological diagnosis and differential diagnosis of the infantile hemangiopericytomas （HPC）. Methods The clinical pathological data and immunohistochemical findings of 9 cases of HPC were retrospectively analyzed in combination with review of the relevant literature. Results Six cases were male and 3 cases were female. The median age of patients were 1.6 months old. The main clinical presentation was local mass. The average size of tumor was 4. 63 cm. 8 cases were diagnosed as benign, only 1 case was low grade malignant. 7 cases were followed up for 2-36 months, without tumor recurrence and metastssis. Histologically, the tumor was composed of variably sized ectatic vessels showing a staghorn configuration and closely packed ovoid or spindle cells. The tumor cells had a high mitotic rate （ more than 4 per 10 high power fields ）. Necrosis and calcification were also presented in some cases. Immunohistoehemically, all of the tumors were positive for vimentin, most of them were positive for CD34, CD31, factor Ⅷ, CD99, BCL-2 and actin. Ki-67 proliferation index was from 5% to 30% , with a median of 12%. Conclusion Infantile HPC is a rare soft tumor, and it is difficult to diagnose and identify from other tumors before operation. The pathological diagnosis is the most reliable method for differential diagnosis of infantile HPC.

关 键 词：婴儿 血管外皮细胞瘤 临床病理学 免疫组化 

分 类 号：R732.2[医药卫生—肿瘤]