title: The role of (18)F-FDG PET/CT imaging in patient with malignant PEComa treated with mTOR inhibitor.
abstract: Malignant perivascular epithelioid cell tumor (malignant PEComa) is a rare disease for which the diagnostic criteria and treatment options have not been established. Since PEComa is associated with upregulation of mammalian target of rapamycin (mTOR) pathway which controls Glut-1 (glucose transporter) function, increased (18)F-fluorodeoxyglucose ((18)F-FDG) uptake may indicate the over activation of mTOR pathway and may guide selectively inhibiting mTOR pathway treatment. We report a malignant PEComa patient who presented for (18)F-FDG positron emission tomography/computed tomography (PET/CT) restaging. The tumor had shown significant avidity on PET/CT as well as an evident response to sirolimus (rapamycin, Rapamune™) that supports the utility of mTOR inhibitors as an effective treatment for malignant PEComa. Therefore, (18)F-FDG PET/CT is helpful in restaging and guiding treatment for malignant PEComa with mTOR inhibitors.
authors.affiliation: School of Medicine and Life Sciences, University of Jinan-Shandong Academy of Medical Sciences, Jinan, Shandong, People's Republic of China.
authors.forename: Lu
authors.initials: L
authors.lastname: Sun
authors.affiliation: PET/CT Center, Department of Radiology, Shandong Cancer Hospital and Institute, Jinan, Shandong, People's Republic of China.
authors.forename: Xiaorong
authors.initials: X
authors.lastname: Sun
authors.affiliation: Department of Pathology, Shandong Cancer Hospital and Institute, Jinan, Shandong, People's Republic of China.
authors.forename: Yuhui
authors.initials: Y
authors.lastname: Li
authors.affiliation: Department of Radiation Oncology, Shandong Cancer Hospital and Institute, Jinan, Shandong, People's Republic of China.
authors.forename: Ligang
authors.initials: L
authors.lastname: Xing
keywords: FDG; PEComa; PET/CT; mTOR inhibitor; malignant perivascular epithelioid cell tumor