Although mTOR inhibitors are effective in the treatment of pulmonary and renal LAM, (US FDA approved), there are limited studies which demonstrate their efficacy in extrapulmonary LAM, as in our patients.[12,13,22] To our knowledge, only 2 published articles have reported the effects of mTOR inhibitors on extrapulmonary LAM. This evidence concerns the gene MTOR and lymphangioleiomyomatosis.