· To assess the clinical characteristics and treatment strategies of Kaposi form hemangioendothelioma (KHE) in children.

· The retrospective study was carried out on 19 cases of children diagnosed with KHE from January 2009 to December 2017 in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine.The general demographics, tumor characteristics, presence or absence of Kasabach-Merritt phenomenon (KMP), treatment and outcome were statistically analyzed. The difference between the children with KMP and without KMP were compared in whether the tumor involved the skin and the deep muscles.

· Of the 19 children with KHE, 13 (68.4%) were male and 6 (31.6%) were female; 17 children (89.5%) presented within the first year of life and 12 children (63.2%) were detected at birth; 6 cases occurred on head and neck, 4 cases in the trunk, 9 cases in the extremities, and none was multifocal.Seven cases (36.8%) were complicated with KMP after onset. There was no significant difference in cutaneous lesions (P=0.617), while there was a significant difference in deep muscles involvement (P=0.017) between the cases complicated with KMP and without KMP. All cases were diagnosed on the basis of histopathology and immunohistochemistry after operation, in which 8 were completely removed, 5 were partially excised, and 6 were performed biopsy. All the tumors were substantial and aggressive growth. Seven children were treated by propranolol before operation but no obvious effect was found. Ten children were given oral corticosteroids, and five of them were found tumor shrinking and underwent operation. The treatment of corticosteroids resulted in higher effect than that of propranolol (P=0.044). Two children who showed resistance to corticosteroids accepted a combination therapy of corticosteroids and vincristine, and the tumor shrank at last. During the follow-up of 2 to 5 years, the cases which performed complete resection were without recurrence, and those who accepted partial excision also survived with postoperative corticosteroids treatment.

· Most KHE cases occur in infancy. The cases might be predisposed with KPM when the tumors involve the deep muscles. The children with typical clinical features should undergo operation as soon as possible. Besides, those with atypical characters should take biopsy to achieve definite diagnosis. The cases with KMP or with giant tumors that are impossible to be removed could be given corticosteroids treatment with or without vincristine. Although propranolol showed a poor curative effect, it could be used for a diagnostic treatment.