Purpose of review: Individuals that present with renal cell carcinoma at an early age deserve special attention because they are more likely to harbor an inherited predisposition and may require unique treatment strategies.
Recent findings: In recent years, there has been a significant increase in the number of characterized hereditary syndromes, many of which may predispose to kidney cancer. Although hereditary kidney cancer accounts for only 5-8% of kidney cancers, it should be considered in anyone with an early age of onset. These uncommon presentations should prompt consideration for genetic evaluation for germline alterations or somatic alterations associated with translocations. Treatment of kidney cancer in young individuals must also consider the late risks of nephron loss, the uncertainty with long-term follow-up after ablation, and the need for years of surveillance not only for the treated tumor but for de novo lesions in a patient with a greater risk of multifocality.
Summary: Early age of onset should raise concern for a hereditary renal cell carcinoma syndrome and should prompt consideration for genetic counseling to review personal and family history and determine if testing is indicated. Despite advancements in this field, significant work remains to fully delineate the management strategies for young individuals with and without hereditary cancer syndromes.