TSC1 and neoplasm with perivascular epithelioid cell differentiation: For TSC-1 or TSC-2 mutated PEComa treated with mTOR inhibition as first-line therapy, the ORR was 25% and CBR was 75% compared to 33% and 66%, respectively, for PEComa without the TSC-1 or -2 mutations also treated with mTOR inhibition (Table 2).