New and emerging renal tumors

Abstract

The International Society of Urological Pathology (ISUP) 2012 Consensus Conference on Adult Renal Neoplasia made recommendations regarding the diagnosis and classification of new and emerging adult renal tumors. There was consensus that five entities should be recognized as new histological types: tubulocystic renal cell carcinoma (RCC), acquired cystic disease-associated RCC, clear cell (tubule) papillary RCC, MiT family translocation RCC [in particular t(6;11) RCC] and hereditary leiomyomatosis-associated RCC. In addition, three rare epithelial carcinomas were considered emerging or provisional entities: thyroid-like follicular RCC; succinate dehydrogenase B deficiency-associated RCC; and ALK-translocation RCC. There were also a number of suggested modifications to existing WHO 2004 categories with the new classification. This new classification of renal neoplasia is known as the ISUP Vancouver Classification. These new renal tumor types have clinicopathological and molecular features that are distinct from those included in 2004 WHO classification. Correct diagnosis and classification of these new renal tumor entities have important implications for patient management.