Hereditary renal cancer syndromes

Despite the vast majority of renal cancers being sporadic, there are a number of conditions which increase the risk of renal cell cancer.  They include

• von Hipple Lindau syndrome (vHL) - predominantly clear cell type
• tuberous sclerosis (TS) - predominantly clear cell type
• hereditory leiyomyomata renal cell cancer syndrome (HLRCC) -Hereditary hereditary leiomyomatosis RCCSyndrome–associated syndrome-associated RCC (newly described entity in ISUP Vancouver classification³ )
• Birt-Hogg-Dube syndrome - predominantly chromophobe renal cell cancer (also associated with oncocytic adenomas)
• hereditory papillary renal cell cancer type 1 - papillary type I
• hereditory non polyposis colon cancer (HNPCC) (Lynch type II) - predominantly transitional cell carcinoma of renal pelvis
• hereditory renal oncocytoma