Abstract
Perivascular epithelioid cell tumours (PEComas) are rare mesenchymal tumours. Some have a benign course; others metastasize. Treatment of malignant PEComas is challenging, and little is known about treatment for patients with metastatic disease. Here, we report a case of metastatic malignant PEComa with estrogen and progesterone receptor expression that showed a favourable and sustained response to letrozole.