A comparative study on the clinical features of kaposiform hemangioendothelioma and tufted angioma
10.3760/cma.j.cn114453-20210513-00214
- VernacularTitle:卡波西样血管内皮瘤与丛状血管瘤临床对比研究
- Author:
Shiyi DAI
1
;
Xuepeng ZHANG
;
Jiangyuan ZHOU
;
Tong QIU
;
Kaiying YANG
;
Siyuan CHEN
;
Yi JI
Author Information
1. 四川大学华西医院小儿外科，成都 610041
- Keywords:
Hemangioendothelioma;
Kasabach-Merritt syndrome;
Tufted angioma
- From:
Chinese Journal of Plastic Surgery
2022;38(3):284-290
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the similarities and differences in clinical manifestations, lesion features, treatment options and prognosis in patients with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA).Methods:The clinical data of KHE patients and TA patients diagnosed and treated in West China Hospital of Sichuan University from August 2016 to June 2020 were retrospectively analyzed. The similarities and differences in gender, the age of onset, clinical features, complications, histopathology, imaging manifestations, treatment and prognosis of the two diseases were compared. The χ2 test or Fisher’s exact test were used to analyze the qualitative data. The Mann-Whitney U test was used to analyze the quantitative data of the two groups that did not conform to the normal distribution. P<0.05 was considered to be statistically significant. Results:A total of 217 patients were included, including 183 patients with KHE and 34 patients with TA. There was no significant difference between KHE and TA in male to female ratio ( χ2=0.44, P=0.510), the age of onset ( U=2 757.00, Z=-1.09, P=0.278) and the sites of lesion ( χ2=3.64, P=0.162). The mixed type of KHE was the most common, reaching 63.39% (116/183), while the superficial type of TA was the most common, reaching 88.24% (30/34). The lesion diameter of KHE patients was 6.30(4.40, 9.70) cm, which was larger than that of TA patients 2.95(2.05, 4.03) cm, and the difference was statistically significant ( U=967.50, Z=-6.38, P<0.001). KHE is more likely to involve skeletal muscle, and cause thrombocytopenia and severe fibrinogenopenia. KHE mainly involved the dermis, subcutaneous tissue and even deep muscles, and manifested as an infiltrative mass. Magnetic resonance imaging (MRI) showed mass diffuse with high signal on T 2 phase. TA was often a superficial lesion that only involved the subcutaneous fat layer and was lumpy. MRI showed that the high signal was confined to the subcutaneous fat layer. The total effective rate of KHE [KHE(85.79%) vs. TA(91.18%)] and the total effective rate of drug therapy [KHE(85.32%) vs. TA(95.65%)] were lower than those of TA. Conclusions:Compared with TA, KHE has a larger tumor diameter, higher invasiveness, higher risk of complications. In addition, treatment plan was more complicated and treatment response rate was lower in patients with KHE compared with those in patients with TA.
