Advanced alveolar soft part sarcoma responds to apatinib

// Yong Zhou 1,* , Fan Tang 1,2,* , Yiying Wang 3 , Li Min 1,2 , Yi Luo 1 , Wenli Zhang 1 , Rui Shi 1 , Hong Duan 1 and Chongqi Tu 1 1 Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, China 2 Sarcoma Biology Laboratory, Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital, Boston, MA, USA 3 Department of Pathology, West China Hospital, Sichuan University, Chengdu, China * These authors have contributed equally to this study Correspondence to: Chongqi Tu, email: // Keywords : alveolar soft part sarcoma, vascular endothelial growth factor receptor-2, anti-angiogenesis, chemotherapy resistance, apatinib Received : January 28, 2017 Accepted : June 08, 2017 Published : June 22, 2017 Abstract Alveolar soft part sarcoma (ASPS) is a rare, hypervascular soft tissue sarcoma with a low chemotherapy response rate. Here, we report an ASPS case with multiple lung metastases on initial presentation. The primary tumor, a hypervascular soft tissue mass 4.1×3.2×2.0 cm, located in the right thigh, was resected prior to chemotherapy. The patient suffered disease progression after two cycles of gemcitabine-docetaxel treatment. Immunohistochemical examination of the tumor tissue revealed strong positive staining for vascular endothelial growth factor (VEGF) and VEGF receptor-2 (VEGFR-2). The patient was subsequently treated with apatinib (500 mg/day), a specific VEGFR-2 inhibitor. Treatment was well tolerated, and the patient exhibited a partial response, with the lung metastases reduced in size and number after one month of therapy. To date, 12-month progression-free survival has been achieved. Apatinib may provide an additional treatment option for metastatic ASPS, particularly in cases resistant to other chemotherapeutic options. Furtherstudies with more cases with longer follow-up times will be necessary to determine the clinical efficacy of apatinib for treatment of ASPS.