45-year-old male, abdominal tumour and increasing renal failure with recurrent haematuria. Positive family history. Nephrectomy in preparation for transplantation.
-Large cysts lined by cuboid or flat cells (often desquamated).
- In between the remaining atrophic renal tissue, partly with accompanying inflammation.
- Extensive scarred tissue and signs of older bleeding: cholesterol crystals with foreign-body reaction and macrophages filled with haemosiderin.
Autosomal dominant tubular structure defect. Symptoms typically do not occur until middle age.
Often giant kidneys which can cause space problems.
Complications: Frank haematuria which is occasionally an indication for nephrectomy.