Clinical and imaging findings of Kaposiform hemangioendothelioma involved bone
10.13929/j.issn.1003-3289.2024.09.003
- VernacularTitle:卡波西型血管内皮瘤累及骨骼:临床及影像学表现
- Author:
Qionghe LIANG
1
;
Peng ZHANG
;
Ming YANG
;
Hongmei GUAN
Author Information
1. 南京医科大学附属儿童医院放射科,江苏南京 210008
- Keywords:
hemangioendothelioma;
bone and bones;
diagnostic imaging
- From:
Chinese Journal of Medical Imaging Technology
2024;40(9):1289-1293
- CountryChina
- Language:Chinese
-
Abstract:
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 type Ⅰ(confined to only bone)and type Ⅱ(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 type Ⅰ and Ⅱ were 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 type Ⅰ,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 type Ⅱ,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 type Ⅱ lesion(2/3)on enhanced CT,while no obvious enhancement was found in 1 case with type Ⅰ lesion(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,while the affected soft tissue were found with equal T1 and slightly higher T2 signal.Conclusion Clinical and imaging findings of KHE involved bone had certain characteristics.