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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

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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T1WI呈等和低信号,T2WI以高信号为主,伴条带状、斑点状低信号,T,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 hemangioendothe-lioma(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 lo-cated in trunk,2 cases in head and neck,l 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 T1WI showed iso-intensity and low-intensity,T2 WI fat-suppressed mainly hyper-intensity,accompanied by banded and spot-like low-intensity T1WI 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 o-val or spindle-shaped endothelial cells and capillaries were nest-like hyperplasia,resembling"glomeruli",immunohistochemis-tryshowed 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.

Kaposiform hemangioendotheliomaKasabach-Merritt phenomenonPathologyTomography,X-ray compu-tedMagnetic resonance imagingInfants

陈希文、陈臻、吴慧莹、鹿连伟、周宁、徐文彪、蔡培珊

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510623 广州市妇女儿童医疗中心放射科,广东省儿童健康与疾病临床医学研究中心

卡波西样血管内皮瘤 卡梅现象 病理 体层摄影术,X线计算机 磁共振成像 婴幼儿

2024

临床放射学杂志
黄石市医学科技情报所

临床放射学杂志

CSTPCD北大核心
影响因子:0.872
ISSN:1001-9324
年,卷(期):2024.43(2)
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