I3. Documented disease progression at the time of study entry. I4. At least one prior systemic treatment regimen for locally advanced or metastatic disease. Patients who are candidates for a validated second line treatment regimen are not eligible for the study. For patients with a primitive CNS tumor, the absence of other therapeutic options must be validated by the reference committee for the patient's pathology before inclusion. As there is no prior systemic treatment regimen available for locally advanced or metastatic PEComa, these tumors are eligible for a MTT treatment in first line of their advanced or metastatic disease I5. Patient with measurable disease, defined as at least one lesion that can be accurately measured on CT-scan or MRI according to RECIST 1.1 I6. A multidisciplinary molecular board must have recommended one of the investigational MTT available in the study after review of a tumor molecular profiling previously established from a biopsied lesion and/or primitive tumor.