Background and objective: Kaposiform hemangioendothelioma (KHE) is a rare, aggressive and borderline vascular tumor mainly occurring in infants and children. The aim of this study was to determine the health-related quality of life (HRQOL) in children with KHE.
Methods: A total of 91 children with KHE participated in this cross-sectional study. The HRQOL was assessed by the age-specific Pediatric Quality of Life Inventory Version 4.0 (PedsQL™ 4.0) Infant Scales, Family Information Form (FIF), Family Impact Module (FIM) and Generic Core Scales (GCS). For comparison, demographically matched healthy children were recruited as a control group. The main outcome measure of HRQOL was analyzed in the two groups. We determined related factors that influenced the HRQOL in children with KHE and their parents by using a stepwise multiple regression analysis.
Results: Except for social and cognitive functioning, we found significant differences in the PedsQL™ 4.0 Infant Scales subscales between the patient group and healthy group (P<0.05). In the PedsQL™ 4.0 GCS, all the subscales were significantly different between the patient group and the healthy group (P<0.05). Additionally, in the ≤24 month age group, there were significant differences in the HRQOL between patients with and without Kasabach-Merritt phenomenon (KMP) in physical, physical symptoms, emotional and cognitive functioning (P<0.05). In the >24 month age group, physical, emotional and social functioning were significantly different between the patients with and without activity dysfunction (P<0.05).
Conclusions: The findings presented here suggest that patients with KHE have a poor HRQOL. KMP and activity dysfunction are risk factors for poor HRQOL in patients with KHE. However, lesion size, lesion location and education level of the mother and father were not related to the HRQOL.