Paradoxical to its role in maintaining hypoxic cell viability, mutations or loss of essential autophagy genes including ATG2B, ATG9B, UVRAG (UV radiation resistance associated) and BECN1 (beclin 1) have been reported in gastric, colorectal, renal cell carcinoma, breast- and ovarian cancer [19–21] and are considered to have supported tumorigenesis. Here, BECN1 is linked to hereditary clear cell renal cell carcinoma.