Abstract: Uterine perivascular epithelioid cell tumor is a clinically rare mesenchymal tumor, and its biological behavior is still uncertain, and malignantcy is rare. The incidence of such diseases is low, the cause is not clear, the clinical and imaging findings are not typical, and it is more common in middle-aged and older women. It is often found because of abdominal mass in physical examination. Usually, the immunophenotype can have characteristic dual expression: simultaneous expression of melanin specific antibody HMB45 and myogenic marker SMA. Recent studies have found that translocation factor E3 (TFE3) is associated with the occurrence of disease. Folpe divides it into benign, malignant potential and malignant according to its degree of risk. The disease is difficult to diagnose before surgery, and it is easy to be confused with uterine epithelioid leiomyoma, low-grade endometrial stromal sarcoma, malignant melanoma and clear cell sarcoma. At present, there is no standardized treatment plan at home and abroad. In actual clinical work, surgical treatment is the main treatment, and complete resection of the tumor is an effective treatment. The effect of radiotherapy and chemotherapy is uncertain, and long-term follow-up should be followed after surgery.

Key words: Perivascular epithelioid cell neoplasms, Uterine neoplasms, Immunophenotyping, Diagnosis