MALIGNANT CERVICAL PECOMA: A CASE REPORT AND REVIEW OF THE LITERATURE

Abstract

Background: Perivascular epithelioid cell tumors represent a family of mesenchymal tumours characterised by co-expression of melanocytic and muscle markers. The PEComas family of tumours includes angiomyolipoma, lymphangioleiomyoma, clear cell "sugar" tumour of the lung and rare clear cell tumours of various location. The designation PEComa, not otherwise specified, has been applied to these "unusual clear cell tumours" which are rare and mainly located in the uterine body. Most PEComas follow a benign clinical course, but tumours with aggressive behaviour have been increasingly reported. Folpe first proposed criteria for assessment of malignancy in 2005. To the best of our knowledge, 35 uterine non-benign and only 13 cervical PEComas have been reported in the available medical literature in the English language. Case description: We report a case of 57-year-old woman with malignant cervical PEComa, emphasising the diagnostic challenges. We have tried to evaluate the malignant potential of uterine PEComas according to Folpe criteria.

Keywords

• pecoma
• smooth muscle epithelioid tumour
• tuberous sclerosis complex