Hormonal Manipulation with Letrozole in the Treatment of Metastatic Malignant pecoma

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.