Renal Replacement Lipomatosis: From Conception to Birth

Abstract

Renal replacement lipomatosis is a rare disease characterized by renal parenchymal atrophy and proliferation of inflammatory and fatty cells within the renal sinus, hilum, and perirenal region. The exact pathogenesis is unknown, however more than 75% of cases are associated with renal stones. Here, we provide images from a case with 10 years of follow-up, outlining the radiographic progression of the disease through time. Although only histopathology can confirm the diagnosis, high clinical suspicion in combination with the radiographic findings can differentiate the disease from other fat-containing entities.