Perivascular epithelioid tumours (PEComas) of Gynaecological Origin (2528)
PEComas are rare tumours of the gynaecological tract only recognised and described in the last 20 years.
These tumours occur at many sites and are characterised by the presence of an epitheloid cell of mixed myomelanocytic immunophenotype. Whilst the exact cell of origin has not been unequivocally established, embryonal studies suggest a neural-crest stem cell that is capable of myoid and melanocytic differentiation. The tumour family includes previously recognised entities such as angiomyoplioma, clear cell ‘sugar’ tumours of the lung and lymphangioleiomyomatosis. This talk aims to highlight the current knowledge of PEComas of extra-gynaecological tract origin including histology, morphology, immunohistochemistry and genetics and then to summarise all reported cases of gynaecological PEComas to date. It will discuss some of the issues with PEComa prognostic classification and examine the performance of several prognostic classification systems for malignancy in these tumours.