Tubulocystic carcinoma of the kidney with fatal outcome in an adolescent male.

Non-clear cell renal cell carcinoma accounts for about 20% of all kidney cancers. Tubulocystic carcinoma of the kidney (TCK) is a newly described entity with distinct molecular, genetic and clinical features. In 2013, it was one of five new renal tumors to be recognized by the International Society of Urological Pathology Vancouver Classification of Renal Neoplasia. We describe here a case of TCK with a rare genetic signature, unusual metastases and limited response to an anti-vascular endothelial growth factor inhibitor in what we believe is the youngest patient on record to date.