Background and Aims:Perivascular epithelioid cell neo-plasms(PEComas)are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging.In this study,we summarized the experience from a single me...Background and Aims:Perivascular epithelioid cell neo-plasms(PEComas)are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging.In this study,we summarized the experience from a single medical center to study the examinations,clinical presen-tations,and pathological and histological characteristics of PEComas in the liver in order to optimize overall un-derstanding of the diagnosis and treatment of these neo-plasms.Methods:We conducted a retrospective analysis to investigate the clinical and pathological characteristics as well as imaging presentations of 75 patients diagnosed with hepatic PEComa in The First Affiliated Hospital of Zhe-jiang University between April 2010 and April 2020.Re-sults:Among the 75 patients,52 were women,and the median age was 48 years.Most patients had no specific symptoms,and two were admitted to the hospital for a second time owing to relapse.All patients underwent surgi-cal resection.Histologically,38 patients had classical angio-myolipoma(AML)and 37 had epithelioid AML.The PECo-mas were accompanied by positive immunohistochemical expression of HMB45,Melan-A,and smooth muscle actin.Follow-up data were obtained from 47 of the total 75 pa-tients,through October 2020.Two patients had metastasis after surgery.Conclusions:AML is the most common type of hepatic PEComa.There are no specific symptoms of he-patic PEComa,and serological examinations and imaging modalities for accurate preoperative diagnosis are lacking.Epithelioid AML should be considered a tumor of uncertain malignant potential;however,the prognosis of PEComa af-ter resection is promising.展开更多
基金This work was supported by Zhejiang Natural Science Foundation(LQ20H030005)Zhejiang Health Technology Project(2020KY126/2019RC153).
文摘Background and Aims:Perivascular epithelioid cell neo-plasms(PEComas)are a rare type of mesenchymal neo-plasm and their preoperative diagnosis is challenging.In this study,we summarized the experience from a single medical center to study the examinations,clinical presen-tations,and pathological and histological characteristics of PEComas in the liver in order to optimize overall un-derstanding of the diagnosis and treatment of these neo-plasms.Methods:We conducted a retrospective analysis to investigate the clinical and pathological characteristics as well as imaging presentations of 75 patients diagnosed with hepatic PEComa in The First Affiliated Hospital of Zhe-jiang University between April 2010 and April 2020.Re-sults:Among the 75 patients,52 were women,and the median age was 48 years.Most patients had no specific symptoms,and two were admitted to the hospital for a second time owing to relapse.All patients underwent surgi-cal resection.Histologically,38 patients had classical angio-myolipoma(AML)and 37 had epithelioid AML.The PECo-mas were accompanied by positive immunohistochemical expression of HMB45,Melan-A,and smooth muscle actin.Follow-up data were obtained from 47 of the total 75 pa-tients,through October 2020.Two patients had metastasis after surgery.Conclusions:AML is the most common type of hepatic PEComa.There are no specific symptoms of he-patic PEComa,and serological examinations and imaging modalities for accurate preoperative diagnosis are lacking.Epithelioid AML should be considered a tumor of uncertain malignant potential;however,the prognosis of PEComa af-ter resection is promising.
