The proportion of patients with PEComa-NOS and TSC is not as large as that linking TSC and LAM or AML [7], but genetic analysis of multiple PEComa-NOS specimens have demonstrated activation of the mTOR pathway [79, 80], indicating potential benefit for mTOR inhibition in PEComa-NOS. Here, MTOR is linked to neoplasm with perivascular epithelioid cell differentiation.