Perivascular Epithelioid Cell Tumors (PEComas) Refractory to mTOR Inhibitors

Perivascular epithelioid cell tumors (PEComas) are mesenchymal tumors with a particular perivascular epithelioid cell differentiation. These tumors are extremely rare and represent a form of malignancy with certain characterizations. The TSC1/2 gene mutation can develop in both tuberous sclerosis complex (TSC)-related PEComa and sporadic cases. The mTORC1 pathway activation is also found in these tumors. Currently, mTOR inhibitors have been used for the treatment of PEComas, and some reports have shown durable responses with the use of such mTOR inhibitors. We present a 71-year-old woman who had recurrent PEComa which was refractory to temsirolimus and everolimus