Hormonal manipulation with letrozole in the treatment of metastatic malignant PEComa

P. Le, A. Garg, G. Brandao, A. Abu-Sanad, L. Panasci


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.


Malignant pecoma; pecomal; receptors; estrogen; progesterone; metastasis; letrozole; sirolimus

Full Text:


DOI: http://dx.doi.org/10.3747/co.21.1849

Copyright © 2019 Multimed Inc.
ISSN: 1198-0052 (Print) ISSN: 1718-7729 (Online)