HEREDITARY PAPILLARY RENAL-CELL CARCINOMA

We describe a 3 generation family with members affected with papillary renal cell carcinoma, an uncommon histological type of renal cell car cinoma. Multiple tumors of varying size were present in both kidneys o f affected family members. The disorder was not linked to polymorphic markers on chromosome 3p and there was no loss of heterozygosity at lo ci on 3p in renal tumors. The results suggest the presence of a renal cell carcinoma gene not located on 3p that predisposes to renal cell c arcinoma with a distinct histological appearance. The inherited disord er in this family appears to be different from recognized hereditary c ancer syndromes.