TFE3-expressing malignant perivascular epithelioid cell tumor of the mesentery:A case report and review of literature  

作　　者：Nah Ihm Kim Ji Shin Lee Yoo Duk Choi U Chul Ju Jong Hee Nam 

机构地区：[1]Department of Pathology,Chonnam National University Hospital and Medical School,Gwangju 61469,South Korea [2]Department of Pathology,Chonnam National University Hwasun Hospital and Medical School,Hwasun 58128,South Korea [3]Department of Obstetrics and Gynecology,Chonnam National University Hwasun Hospital and Medical School,Hwasun 58128,South Korea

出　　处：《World Journal of Clinical Cases》2020年第18期4207-4214,共8页世界临床病例杂志

摘　　要：BACKGROUND Perivascular epithelioid cell tumor(PEComa)is a rare mesenchymal tumor that exhibits an epithelioid and spindle cell morphology.The tumor is characterized by immunoreactivity for melanocytic and myogenic markers but can be misdiagnosed as more common tumors with similar characteristics,including gastrointestinal stroma tumors or leiomyosarcomas.Recently,a subset of PEComas has been reported to harbor a transcription factor binding to TFE3 fusion.Herein,we report a rare case of TFE3-expressing malignant PEComa arising from the mesentery.CASE SUMMARY A 50-year-old woman presented with abdominal discomfort for 3 months.Results of laboratory tests were all within the normal ranges,and the patient had no notable medical history.Magnetic resonance imaging revealed a large tumor on the right side of the pelvic floor,which was originally suspected to be a primary ovarian tumor.However,during surgery,the tumor was revealed to have originated from the mesentery.Histologically,the tumor was composed of bundles of spindle cells and sheets of epithelioid cells.Extensive coagulative necrosis and numerous mitotic figures were observed.Immunohistochemistry revealed that the tumor cells were positive for smooth muscle actin,HMB-45,and TFE3 expression.Tumor involvement of the rectal serosa was identified,leading to a final diagnosis of malignant PEComa of the mesentery.Surgical resection was followed by adjuvant chemotherapy.No recurrence or metastasis was observed over a 6-month follow-up period.CONCLUSION Malignant PEComa of the mesentery is extremely rare and should be distinguished from morphological mimics through differential diagnosis and immunohistochemistry.

关 键 词：Perivascular epithelioid cell tumor TFE3 Differential diagnosis MESENTERY HISTOLOGY Case report 

分 类 号：R735.3[医药卫生—肿瘤]