To summarize and analyze the clinical characteristics of Kaposiform hemangioendothelioma (KHE) in infants and explore the individualized treatment strategies.

The clinical data of 20 infants with KHE treated in Jiangxi Provincial Children's Hospital from January 2016 to March 2020 were selected and analyzed. The general demographics, tumor characteristics, presence or absence of Kasabach-Merritt phenomenon (KMP), treatment strategies and outcome of the cases were retrospectively analyzed. The differences in clinical features between children with KMP and without KMP were analyzed.

A total of 20 infants with KHE were enrolled, including 6 females (30.0%) and 14 males (70.0%). Sixteen infants (80.0%) were younger than 1 year old, and 13 of them (65.0%) were found at birth. There were 8 infants with KMP, and there were statistically significant differences in tumor size (P=0.009) and invasion of deep muscle (P=0.003) between children with and without KMP. Among the children with KMP, 3 cases were treated with mesh suture, and the other 5 were given oral sirolimus. 1 case was treated with oral sirolimus after mesh suture therapy failed. Among the 12 infants without KMP, 7 received complete tumor resection, 2 received partial tumor resection combined with oral glucocorticoid, 2 received mesh suture therapy, and 1 received oral sirolimus. 1 case was treated with oral sirolimus after mesh suture therapy failed. The patients were followed up for 2-5 years after treatment. Among the infants with KMP, the tumor was nearly disappeared in 1 case, and 7 cases survived with tumor. In the infants without KMP, 7 cases of complete tumor resection were effective and no recurrence was observed, and 5 cases achieved asymptomatic survival with tumor.

Most of KHE cases are in infancy, and the size of tumor and the involvement of the lesion in the deep muscle are related to KMP. For infants complicated with KMP, initial high-dose glucocorticoid implosive therapy or infusion of blood products to stabilize the disease, suitable lesions can be treated with mesh suture first. If the lesion is not suitable for mesh suture or does not respond to treatment, oral sirolimus alone or in combination may be used.