Objective: To explore the clinicopathologic features of kaposiform hemangioendothelioma (KHE).
Methods: The clinicopathologic data were studied in three cases of KHE and review the literatures.
Results: Two cases were female and one was male. All cases occurred in infancy. Two tumor located in axillary chest wall and one in lumbar region. All of the three patients had Kasabach-Merritt syndrome. Histologically, the tumor was composed of spindle-shaped cells. in all cases nodular growth pattern was seen. Immunohistochemically, Neoplastic spindled cells expressed CD34 and CD31. Associated lymphangiomatosis was present in two cases. Two tumors were resected completely, one was resected partly. the follow-up period ranged from 6 months to 3 years, and all were alive.
Conclusions: Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor that mainly occurred in early infancy. It is frequently complicated by Kasabach-Merritt syndrome, and it has features common to both capillary hemangioma and Kaposi sarcoma. The prognosis of KHE is determined by the size, location and the hemorrhage degree of vascular tumor. Better outcome might be achieved in patients with KHE of the skin and in the soft tissues under the skin. It appears that the main treated measure should be wide local excision.