部分消退型先天性血管瘤的临床病理分析

Clinical and Histopathologic Features of Partially Involuting Congenital Hemangioma

目的探讨部分消退型先天性血管瘤(Partially involuting congenital hemangioma,PICH)的临床特点及病理特征。方法回顾7例PICH患者的临床及病理资料,分析其特点及与其他先天性血管瘤的异同。结果本组中男性2例,女性5例。病灶位于肢体5例,位于躯干2例。病灶半球形,质地偏硬,周围有淡色晕环。病灶最大直径均大于5 cm。出生后即开始消退,消退时间最短7周,最长48周,平均28周。消退后残留瘤体表面可见扩张紫红色毛细血管,周围有淡色晕环。多普勒超声检查,瘤体为皮下低回声软组织团块,血供丰富,动脉高流速,低阻抗。MRI检查发现肿块均位于皮下浅筋膜层,边界清楚、信号均匀,T1W呈低信号,T2W高信号,增强后肿块明显均匀强化。HE染色,镜下可见真皮及真皮下层增生小血管,形成大小不等小叶状结构,或无明显小叶结构,小叶内充满大小不等的薄壁血管。小叶内部分区域,血管内皮细胞肿胀,胞核大小不一、浓染,核/浆比增大,但无明显异型性,有丝分裂像少见。所有标本中,均可见部分内皮细胞胞核凸向管腔,形成特殊的"钉突样"细胞。免疫组化,全部标本CD34、CD31均为阳性表达,GLUT1均为阴性表达。结论 PICH的临床表现介于RICH和NICH之间,PICH的病理表现与其他先天性血管既有交叉又有区别。消退稳定后,PICH更接近于不消退型先天性血管瘤。深入研究PICH或有助于揭示先天性血管瘤的发病机制。

Objective To study the clinical characteristics and histopathologic features of partially involuting congenital hemangiomas （PICH）. Methods The clinical and histopathologic data of 7 RICH patients were reviewed retrospectively and compared to the non-involuting congenital hemangioma （NICH） and rapidly involuting congenital hemangioma （RICH）. Results Two male and five female healthy infants presented with a vascular tumor involving the extremity （n=5） and trunk （n=3） at birth. The mass was round to ovoid in shape, bossed and solid, pink to purple and surrounded by a pale halo. Size of tumor was over 5.0 cm in diameter （greatest dimension） in all patients. All had rapid involution during the first 7 to 48 weeks （mean 28 weeks） of life before stabilizing in size and appearance. The overlying skin was frequently punctuated by coarse telangiectasia and surrounded by a pale halo. Doppler ultrasound imaging demonstrated hypervascular subcutaneous lesions with low-resistance, high-velocity arterial flow. MRI： the T1-weighted magnetic image showed subcutaneous lesions with uniform signal intensity and the T2-weighted magnetic image showed homogeneously increased signal intensity. T1-weighted magnetic image after gadolinium revealed an intense and homogeneous enhancement with well-defined limits. HE staining：the architecture in most specimens was dominated by lobules of capillaries in the dermis and subcutis that were separated by dense fibrous tissue. The lobules were composed of thin-walled capillaries, usually with round lumina in various size. The endothelial cells were moderately plump and had a dark, round, protruding nucleus. The nucleus/cytoplasm ratio increased, but there was no obviously atypical and the mitoses was rarely seen. The ＂hobnailed＂ endothelial cells lined the small intralobular vessels were observed in all specimens. Immunostaining for CD31 and CD34 demonstrated strong expression in lobular and extralobular endothelial cells. Endothelial cells were negative for GLUT-1 in all stained tissues. Conclusion PICH exhibits ＂transition＂ between the rare RICH and NICH. It has overlapping histologic features with the other two kinds of congenital hemangioma. In PICH, involution is aborted and the residual tumor is indistinguishable from NICH to some extent. The obvious differences in behavior served to differentiate PICH, RICH, NICH, and common infantile hemangioma. Further study on PICH will help to reveal the pathogenesis of congenital hemangioma.