PEComa. Literature review and case report

The family of mesenchymal tumors called PEComas (Perivascular Epithelioid Cell Tumors) has been included in the international Classification of Soft Tissue and Bone Tumors since 2002. Literature review confirms difficulties of timely diagnosis and absence of progress in the treatment results. in spite of advances in research of PEComas histological nature of these tumors is still unclear. Further research of prognostic factors is needed to give proper treatment. Patients with PEComas of different primary sites require close follow-up after the treatment irrespective of malignant potential of tumors, as late recurrence and metastases are possible. This paper presents a case report of 11-year-old girl with vaginal PEComa, who is disease-free 10 years after the treatment.