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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 clinicopathological characteristics, diagnosis and differential diagnosis of infantile hemangiopericytoma (HPC). Methods Nine cases of infantile hemangiopericytoma were retrospectively analyzed. The clinicopathological features, immunohistochemical findings and review of relevant literature were retrospectively analyzed. Results There were 6 male infants and 3 female infants, with a median age of 1.6 months. The main symptoms are local lumps. Mass average diameter of 4.6 cm. 8 cases were benign and 1 case was low grade malignancy. Seven cases were followed up for 2-36 months without recurrence and metastasis. HPC by the rich vascular network and closely arranged around the round or spindle tumor cells, blood vessels expand, different sizes, can be characterized by “antlers” thin-walled branch vessels, cell density, mitosis and more Visible necrosis and calcification. Immunohistochemistry showed that the proliferative index of proliferative index of Ki-67 was 5% -30% with an average of 12% in all cases, including CD34, CD31, Ⅷ factor, CD99, bcl-2 and actin (+). Conclusion Infantile hemangiopericytoma is a rare soft tissue tumor with no characteristic in clinical and imaging studies. Histopathology and immunohistochemistry are the most reliable methods for differential diagnosis.