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By one case of hemangioendothelioma of bone accompanying pulmonary metastasis was treated with rh-enostatin injection (Endostar) combined with chemotherapy. The patient got partial response (PR) for 3 years after the plication of Endostar maintenance therapy and Endostar combined with taxane-based chemotherapy. During the periousing Endostar as monotherapy, the patient got long-term disease control and good quality of life. There was no drug relaadverse event during the therapy of Endostar. Suggested continued using of Endostar combined with chemotherapy coachieve an convinced therapeutic effect. Then using Endostar as maintenance treatment after patient got the optimal efficwas feasible and profitable. This treatment strategy of long-term administration of Endostar was worthy of further observato explore the feasibility for long-term administration of combined with chemotherapy in the treatment of hemangioendoioma of bone accompanying pulmonary metastasis.