婴幼儿卡波西样血管内皮瘤的临床病理及影像特征

Clinicopathological and Imaging Features of Infant Kaposiform Hemangioendothelioma

目的探讨婴幼儿卡波西样血管内皮瘤(KHE)的临床病理及影像特征。方法回顾性分析2017年7月至2022年4月经病理确诊的8例KHE患儿临床病理及影像资料,行CT平扫及增强扫描8例,行MRI平扫及增强扫描3例。结果8例患儿临床均表现为皮下肿物,5例伴皮肤颜色异常,7例合并卡梅现象(KMP)。影像表现为单发肿物,位于躯干5例,头颈部2例,躯干与颈部1例,中位直径8.3 cm,实体肿物伴组织浸润型7例,无实体肿物的组织浸润型1例,混合型5例,深部型3例;CT平扫主要表现为等和稍低密度,增强后不均匀明显强化7例,不均匀轻度强化1例;MRI平扫T_(1)WI呈等和低信号,T_(2)WI以高信号为主,伴条带状、斑点状低信号,T_(1)WI抑脂增强后呈不均匀明显强化;合并骨质破坏6例,瘤周软组织网状淋巴水肿5例,胸腔积液4例,局部皮肤增厚2例。镜下表现为椭圆形或梭形内皮细胞及毛细血管呈“巢团状”增生,形似“肾小球”,免疫组织化学CD31和CD34均呈阳性,GLUT-1均呈阴性,部分ERG与D2-40呈阳性。结论婴幼儿KHE具有一定的的临床与影像学特征,确诊仍需组织病理支持。

Objective To investigate the clinicopathological and imaging features of infants Kaposiform hemangioendothelioma(KHE).Methods The clinicopathological and imaging data of 8 children with KHE confirmed by pathology from July 2017 to April 2022 were retrospectively analyzed,including 8 cases with plain and enhanced CT scans,and 3 cases with plain and enhanced MRI scans.Results All clinically showed subcutaneous tumors,5 cases were accompanied by abnormal skin color,and 7 cases were combined with Kasabach-Merritt phenomenon(KMP).The imaging showed a single tumor,5 cases located in trunk,2 cases in head and neck,1 case in trunk and neck,with a median diameter of 8.3 cm,7 cases of solid central mass with surrounding infifiltrative portions,and 1 case of infifiltrative lesion without defifined areas of solidity.There were 5 cases of mixed type,and 3 cases of deep type;CT plain scan mainly showed equal and slightly low density,7 cases showed heterogeneous obvious enhancement,and 1 case showed heterogeneous mild enhancement;MRI plain scan T_(1)WI showed isointensity and low-intensity,T_(2)WI fat-suppressed mainly hyper-intensity,accompanied by banded and spot-like low-intensity T_(1)WI fat-suppressed enhancement showed heterogeneous and obvious enhancement;6 cases of bone destruction,5 cases of peritumoral soft tissue reticular lymphedema,pleural effusion in 4 cases,local skin thickening in 2 cases.Microscopeshowed oval or spindle-shaped endothelial cells and capillaries were nest-like hyperplasia,resembling“glomeruli”,immunohistochemistryshowed CD31 and CD34 were positive,GLUT-1 were negative,and some ERG and D2-40 positive.Conclusion Infants KHE has certain clinical and imaging features,and the diagnosis still needs histopathological support.