Clinicopathological features and efficacy of perivascular epithelioid cell tumor
Lin Ying, Liu Xin, Zhang Xiaowei, Chen Yong, Wang Chunmeng, Wang Shengping, Wang Jian, Luo Zhiguo
Abstract
ObjectiveTo analyze the clinicopathological characteristics of perivascular epithelioid cell tumor (PEComa) and the efficacy of mTOR inhibitors in the treatment of advanced PEComa.
MethodsFrom 2007 to 2020, medical information of 17 patients diagnosed with PEComa and treated in Fudan University Shanghai Cancer Center were collected. Survival analysis was performed by Kaplan-Meier method. Radiological response was assessed according to RECIST v1.1.
ResultsPEComa mostly occured in middle-aged women. The most common primary sites were pelvis, lung and abdomen. Relapse usually occured within 2 years after radical surgery. Radical surgery was the main treatment for PEComa of limited stage, and remains the main option for those with limited recurrent lesions. Of the 13 patients with advanced malignant PEComa in our cohorts, the objective response rate of mTOR inhibitors in advanced malignant PEComa was 46%, and median progression-free survival was 27.7 months (95%CI: 4.7~50.5 months). 2 patients discontinued mTOR inhibitor treatment due to pneumonitis combined with dyspnea and fever respectively.
ConclusionsFor advanced malignant PEComa, mTOR inhibitors are effective. Lung toxicity of mTOR inhibitor should be monitored.
Key words:
Perivascular epithelioid cell tumor; mTOR inhibitor; Everolimus
Contributor Information
Lin Ying
Department of Medical Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
Liu Xin
Department of Medical Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
Zhang Xiaowei
Department of Medical Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
Chen Yong
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
Department of Bone and Soft Tissue Sarcomas, Fudan University Shanghai Cancer Center Center, Shanghai 200032, China
Wang Chunmeng
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
Department of Bone and Soft Tissue Sarcomas, Fudan University Shanghai Cancer Center Center, Shanghai 200032, China
Wang Shengping
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
Department of Radiology, Fudan University Shanghai Cancer Center, Shanghai 200032, China
Wang Jian
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, China
Luo Zhiguo
Department of Medical Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China
Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, China
