A novel cancer predisposition syndrome in the Eker rat model.

Hereditary renal carcinomas (RCs) develop in virtually all Eker rats by the age of one year. Investigation of extrarenal primary tumors occurring in Eker rats late in life (at 2 years) additionally revealed pituitary adenomas (17/31 = 54.8% (carrier) vs 7/32 = 21.9% (non-carrier siblings of affected animals), P < 0.01), sarcomas of the spleen (21/31 = 67.7% vs. 0/32 = 0%, P < 0.001) and sarcomas (of probable stromal origin) of the uterus (8/17 = 47.1% vs. 0/15 = 0%, P < 0.01). Thus, the Eker rat might provide a novel animal model of cancer predisposition syndromes.