Perivascular epithelioid cell neoplasm (PEComa) of the female genital tract: a systematic review

Perivascular epithelioid cell tumors (PEComa) are rare mesenchymal neoplasms composed by perivascular epithelioid cells that express melanocytic and smooth muscle markers. Most cases are benign, but a small group behaves malignantly. Unfortunately, as they dont<acute accent> appear frequently, reliable criteria to predict malignancy have not yet been established. The PEComas of the female genital tract represent approximately 25% of the PEComas reported in the literature and the most common site of appearance is in the uterine body; less common sites include the cervix, the ovaries, the fallopian tubes, the vagina/vulva or the round or broad ligament. Uterine PEComas are not distinguishable from other uterine tumors, such as leiomyoma and leiomyosarcoma, before an anatomopathological diagnosis is made. Surgery is the most recommended primary treatment, although adjunctive therapy is generally reserved for high-risk cases. However, the best approach is not well established due to the shortage of cases described until now.<br /> This methodical review aims to summarize, according to of current literature, what is known about the etiopathogenesis, clinical and pathological characteristics of PEComas, focusing on the approach to gynecological cases.<br /> (c) 2024 Elsevier Espana, S.L.U. All rights are reserved, including those for text and data mining, AI training, and similar technologies.