Segmental multicystic dysplastic kidney in an adult: usefulness of enhanced CT in excretory phase

We present an adult case of segmental multicystic dysplastic kidney (SMCDK). The patient had a 10 × 6 cm oval-shaped mass consisting of a solid and multilocular cystic component at the right upper renal sinus. The solid component showed gradual and mild enhancement on dual-phase enhanced CT. Excretory-phase CT demonstrated the excretion of contrast medium into the septa and cysts. Dilation of the upper calices and renal pelvis and atretic renal pelvis in the mass were also clearly visualized in the excretory phase. The signal intensity of the solid component was slightly lower than that of the renal parenchyma on T2-weighted images and similar to that of the medulla on diffusion-weighted images. Right nephrectomy showed a multilocular cystic component within the renal sinus and some of the cysts contained blood. The renal pelvis of the upper moiety was blind, as suggested by CT. Microscopically, there were multiple non-communicating small cysts, as shown by CT, and the wall was lined with immature tubule-like cells. In addition, immature tubule-like cells were seen in the septa. Immature mesenchymal cells were seen around the cysts. The solid component mainly consisted of fibroconnective tissue with immature tubule-like cells. The pathological diagnosis was confirmed as SMCDK. Excretion of contrast media into the septum and cystic component might be a characteristic finding of SMCDK in addition to the finding of a multilocular cystic mass in the upper moiety with urinary tract abnormality.