mTOR inhibitors in uterine and extra-uterine malignant PEComas: A multicenter international case series retrospective analysis

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Abstract
Background: Perivascular epithelioid cell tumors (PEComa) are exceedingly rare mesenchymal neoplasms arising from a broad variety of anatomic sites. Within the female genital tract, most frequently PEComas affect the uterus. Over the last few years, the activity of mTOR inhibitors in these neoplasms has been convincingly shown. The aim of this multicenter retrospective analysis was to compare the activity of mTOR inhibitors in uterine versus extra-uterine malignant PEComas.