Case report of everolimus-induced sustained partial response in metastatic renal epithelioid angiomyolipoma

Özet

Epithelioid variant of angiomyolipoma (EAML) is a newly defined entity and a close mimicker of renal cellcarcinoma. 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 totargeted therapies has paved the way. Recently, tyrosine kinase inhibitors and mTOR inhibitors have beenutilised 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, crizotiniband now maintaining a sustained partial response with everolimus.