For the treatment of unresectable tumors, mammalian target of rapamycin (mTOR) inhibitors and vascular endothelial growth factor receptor (VEGFR) inhibitors are used.Generally, PEComa are benign but often can be locally aggressive and malignant, causing bronchial stenosis and spinal invasion, which can cause severe morbidity, including intractable pain and collapse of lungs due to overgrowth of the mass locally. Here, KDR is linked to neoplasm with perivascular epithelioid cell differentiation.