The high presence of macrophages in different CS subtypes argues in favor of macrophages-targeting therapies to achieve therapeutic efficacy in CS, such as the use of CSF1R inhibitors [101,106] or antibodies depleting specifically M2 macrophages or myeloid suppressive cells by targeting the triggering receptor expressed on macrophages 2 (TREM2) [107]. This evidence concerns the gene CSF1R and Cowden syndrome 1.