Abstract
Epithelioid variant of angiomyolipoma (EAML) is a newly defined entity and a close mimicker of renal cell carcinoma. There is a growing body of evidence to suggest its aggressive behavior in terms of local recurrence, metastasis and death. The treatment for this subset of patients has posed challenges for the experts. Chemotherapy plays little active role and so molecular profiling to identify genomic alterations amenable to targeted therapies has paved the way. Recently, tyrosine kinase inhibitors and mTOR inhibitors have been utilised both in adjuvant and neoadjuvant settings and have shown promising results in terms of survival. We present a case of a metastatic epithelioid angiomyolipoma treated sequentially with imatinib, crizotinib and now maintaining a sustained partial response with everolimus.