卡波西型血管内皮瘤累及骨骼:临床及影像学表现  

Clinical and imaging findings of Kaposiform hemangioendothelioma involved bone

作　　者：梁琼鹤[1] 张朋[1] 杨明[1] 管红梅[1] LIANG Qionghe;ZHANG Peng;YANG Ming;GUAN Hongmei(Department of Radiology,Children’s Hospital of Nanjing Medical University,Nanjing 210008,China)

机构地区：[1]南京医科大学附属儿童医院放射科,江苏南京210008

出　　处：《中国医学影像技术》2024年第9期1289-1293,共5页Chinese Journal of Medical Imaging Technology

摘　　要：目的观察卡波西型血管内皮瘤(KHE)累及骨骼的临床及影像学表现。方法回顾性分析10例经手术或活检病理诊断KHE累及骨骼患儿,均接受病变部位X线及CT平扫检查,其中3例接受增强CT、9例接受平扫MR检查,观察临床及影像学表现,并根据CT所见将其分为Ⅰ型(仅骨受累)和Ⅱ型(骨及周围软组织同时受累)。结果10例中,7例单骨、3例多骨受累;Ⅰ、Ⅱ型各5例。5例Ⅰ型中位发病年龄88.0个月,中位病程4.0个月,均为单骨受累,临床主要表现为疼痛,均不伴卡-梅现象(KMP);5例Ⅱ型中位发病年龄5.0个月,中位病程1.0个月,半数(3/5,60.00%)以上累及多骨(n=3),临床多表现为病变部位肿胀、活动受限,可伴KMP(2/5,40.00%)。X线片显示病变骨质密度不均(8/10,80.00%)或呈片状低密度(1/10,10.00%),1例病变骨质未见明显异常(1/10,10.00%)。CT显示受累骨质均呈虫蚀样或溶骨性骨质破坏,多伴外周骨质增生硬化(8/10,80.00%),2例Ⅱ型CT增强扫描呈明显强化(2/3)、1例Ⅰ型未见明显强化(1/3);5例(5/10,50.00%)软组织受累呈边界不清弥漫性稍低密度影,其中2例增强后明显强化。平扫MRI见受累骨质呈等T1或稍低T1、混杂T2或稍高T2信号,受累软组织为等T1稍高T2信号。结论KHE累及骨骼临床及影像学表现具有一定特点。Objective To observe clinical and imaging findings of Kaposiform hemangioendothelioma(KHE)involved bone.Methods Data of 10 children with KHE involved bone diagnosed by surgery or biopsy pathology who underwent X-ray and non-contrast CT examination of the lesion site were retrospectively analyzed,among them 3 received enhanced CT and 9 received non-contrast MR examination.Clinical and imaging findings were observed,and the lesions were classified into typeⅠ(confined to only bone)and typeⅡ(involved both bone and surrounding soft tissue)according to CT findings.Results Single bone involvement was detected in 7 cases(7/10,70.00%),while multiple bone involvements were noticed in 3 cases(3/10,30.00%).CT typeⅠandⅡwere identified each in 5 cases.The median age of onset was 88.0 months,and the median course of disease was 4.0 months in typeⅠ,all involved single bone,and the main clinical manifestations were pain but without Kasabach-Merritt phenomenon(KMP).The median age of onset was 5.0 months and the median course of disease was 1.0 months in typeⅡ,including 3(3/5,60.00%)cases of multiple bone involvements,with swelling and limited movement of the lesion site,and KMP was observed in 2 cases(2/5,40.00%).X-ray shown most of the lesions with uneven bone density(8/10,80.00%)or lamellar low density(1/10,10.00%),while no obvious abnormality was found in 1 case(1/10,10.00%).On non-contrast CT,all affected bones in 10 cases present as worm erosion or osteolytic bone destruction,which could be accompanied by peripheral bone hyperplasia and sclerosis(8/10,80.00%),while significantly enhancement occurred in 2 cases with typeⅡlesion(2/3)on enhanced CT,while no obvious enhancement was found in 1 case with typeⅠlesion(1/3).Soft tissue involvement presented in 5 cases(5/10,50.00%),with borderless diffuse slightly low-density,including 2 cases of lesions significantly enhanced after enhancement.Non-contrast MRI showed that the affected bone presented equal T1 or slightly lower T1,mixed T2 or slightly higher T2 signal,wh

关 键 词：血管内皮瘤 骨和骨组织 诊断显像 

分 类 号：R732.2[医药卫生—肿瘤] R445[医药卫生—临床医学]