Most notably, tRCCs lack alterations in the VHL tumor suppressor gene; this contrasts with clear cell RCC (ccRCC), the most common type of RCC, in which loss of VHL and concomitant activation of hypoxia inducible factor 2 alpha (HIF-2a) are pathognomonic3. The gene discussed is VHL; the disease is nonpapillary renal cell carcinoma.