Immunohistochemical screening for the diagnosis of succinate dehydrogenase-deficient renal cell carcinoma and fumarate hydratase-deficient renal cell carcinoma

Renal tumors represent heterogeneous and diverse group of neoplasms. The 2013 Vancouver classification of renal tumors and the subsequent 2016 WHO classification represent the foundation of our current knowledge on renal tumors (1). Recent advances have significantly contributed to expanding the morphologic, immunohistochemical, molecular, epidemiologic and clinical features of several novel and emerging renal tumors (2). For example, new evidence has recently emerged on two renal entities—succinate dehydrogenase (SDH)-deficient renal cell carcinoma (RCC) and fumarate hydratase-deficient RCC (FH-deficient RCC). The awareness of these novel renal neoplasms is not only essential for practicing pathologists, but also for clinicians and for patient management, because the navigation through this complex and evolving field is a challenging one, even in centers with large volumes of renal tumors. Importantly, the recognition of these novel renal entities rests mainly on their morphologic recognition, with the aid of immunohistochemistry (IHC).