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Combination targeted therapy of VEGFR inhibitor,sorafenib, with an mTOR inhibitor,sirolimus induced a remakable response of rapid progressive Uterine PEComa
Authors:Fang Gao  Chengsuo Huang  Yiping Zhang  Ruirui Sun  Yujie Zhang  Huijun Wang
Institution:1. Department of Medical Oncology, Shandong Cancer Hospital, Shandong Cancer Hospital affiliated to Shandong University, Shandong Academy of Medical Sciences, Jinan;2. Shandong Province, China;3. Department of Medicine, Weihai Guoan Hospital, Weihai, Shandong Province, China;4. Department of Medicine, Weihai Children's Hospital;5. Weihai, Shandong Province, China;6. Department of Oncology, Xinjiang Medical University Hospital, Wulumuqi, Xinjiang Province, China
Abstract:Perivascular epithelioid cell tumor is a rare tumor. To date, there is no consensus of therapy to be recommended for unresectable disease. For a low incidence and a rarely curable disease, the finding of new therapy is essential.

Here we report the first case of a patient with perivascular epithelioid cell tumor whose disease had a rapid progression after surgery and had a rapid remarkable response of combination therapy of a VEGFR inhibitor, sorafenib, with an mTOR inhibitor, sirolimus.

This result may have potential to deliver a new treatment option and inhibiting the mTOR pathway combined with inhibiting the VEGF pathways may be a useful strategy for malignant PEComas.

Keywords:Combination therapy  mTOR inhibitor  perivascular epithelioid cell tumor  sirolimus  sorafenib  VEGFR inhibitor
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