Malignant perivascular epithelioid cell tumors of the uterine body:a clinicopathological features
Objective To study the clinical and pathological features of malignant perivascular epithelioid cell tumor(PEComa)of the uterine body in order to understand the disease better.Methods The data of 5 cases of malignant PEComa of uterine body in our hospital and 12 cases reported in literature were collected.We analyzed and summarize the diagnosis,differential diagnosis and prognostic factors of the disease.Results The age of patients ranged from 23 to 67 years.The main manifestations were abnormal uterine bleeding and(or)pelvic mass,occasionally in the physical examination of accidental discovery of tumors.The tumor was composed of epithelioid and/or spindle cells,which were often radially arranged around thin-walled blood vessels and were characterized by cell atypia,mitosis,tumor necrosis,and vascular invasion.Tumor cells specifically expressed myogenic and melanin markers.All patients received at least total hysterectomy,and 7 patients received postoperative chemotherapy.Follow-up ranged from 5 to 72 months.4 cases recurred,metastasized and died after operation,2 of them received chemotherapy.Conclusion The diagnosis of malignant PEComa of the uterine body is based on histopathology and immunohistochemistry.Tumor resection is the main treatment,but the effect of adjuvant therapy is not clear.Long-term follow-up is the key to early detection of disease progression and treatment.
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