title: Combination targeted therapy of VEGFR inhibitor, sorafenib, with an mTOR inhibitor, sirolimus induced a remakable response of rapid progressive Uterine PEComa.
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.
authors.affiliation: a Department of Medical Oncology , Shandong Cancer Hospital, Shandong Cancer Hospital affiliated to Shandong University, Shandong Academy of Medical Sciences , Jinan ; Shandong Province , China.
authors.forename: Fang
authors.initials: F
authors.lastname: Gao
authors.affiliation: a Department of Medical Oncology , Shandong Cancer Hospital, Shandong Cancer Hospital affiliated to Shandong University, Shandong Academy of Medical Sciences , Jinan ; Shandong Province , China.
authors.forename: Chengsuo
authors.initials: C
authors.lastname: Huang
authors.affiliation: b Department of Medicine , Weihai Guoan Hospital , Weihai , Shandong Province , China.
authors.forename: Yiping
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: c Department of Medicine , Weihai Children's Hospital ; Weihai , Shandong Province , China.
authors.forename: Ruirui
authors.initials: R
authors.lastname: Sun
authors.affiliation: d Department of Oncology , Xinjiang Medical University Hospital , Wulumuqi , Xinjiang Province , China.
authors.forename: Yujie
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: a Department of Medical Oncology , Shandong Cancer Hospital, Shandong Cancer Hospital affiliated to Shandong University, Shandong Academy of Medical Sciences , Jinan ; Shandong Province , China.
authors.forename: Huijun
authors.initials: H
authors.lastname: Wang
authors.affiliation: a Department of Medical Oncology , Shandong Cancer Hospital, Shandong Cancer Hospital affiliated to Shandong University, Shandong Academy of Medical Sciences , Jinan ; Shandong Province , China.
authors.forename: Shu
authors.initials: S
authors.lastname: Zhang
mesh_terms: D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009536:Niacinamide / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000494:pharmacology / Q000627:therapeutic use; D054973:Perivascular Epithelioid Cell Neoplasms / Q000188:drug therapy* / Q000473:pathology; D010671:Phenylurea Compounds / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D020123:Sirolimus / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D000077157:Sorafenib; D058570:TOR Serine-Threonine Kinases; D014594:Uterine Neoplasms / Q000188:drug therapy* / Q000473:pathology
chemical_list: D000970:Antineoplastic Agents; D010671:Phenylurea Compounds; D009536:Niacinamide; D000077157:Sorafenib; C546842:MTOR protein, human; D058570:TOR Serine-Threonine Kinases; D020123:Sirolimus
keywords: Combination therapy; VEGFR inhibitor; mTOR inhibitor; perivascular epithelioid cell tumor; sirolimus; sorafenib