Acquired Cystic Disease

Anthony Chang, MD

Aleksandr Vasilyev, MD, PhD

Key Facts

Terminology

Presence of 3 or more cysts in native kidneys of patients with end-stage renal disease satisfies clinical diagnosis of ACD

Clinical Issues

60-80% of patients on dialysis for more than 4 years may be diagnosed with ACD
- Increased risk of developing renal cell carcinoma
- 2-7% of patients with ACD will develop cystic renal cell carcinoma
Rapid change in cyst size warrants nephrectomy
Renal transplantation may decrease cyst size

Macroscopic Features

Microscopic Pathology

Tubular cysts
- Present throughout cortex and medulla
- Lined by flat cuboidal epithelial cells
- Most stain for proximal tubular markers
- Intracystic hemorrhage may be present
Diffuse interstitial fibrosis and tubular atrophy
Calcium oxalate crystal deposition

Top Differential Diagnoses

Axial CECT shows innumerable cysts in bilaterally enlarged kidneys of a patient who has been on dialysis for many years.

Multiple cysts

are located throughout this end-stage kidney from a dialysis patient. This native kidney was removed for a suspicious mass

, which demonstrated both clear cell and papillary features.

TERMINOLOGY

Abbreviations

Synonyms

Definitions

Presence of 3 or more cysts in native kidneys of patients with end-stage renal disease satisfies clinical diagnosis of ACD

ETIOLOGY/PATHOGENESIS

Unknown Pathogenesis

Believed to involve physiologic response to chronic renal failure
- Possible uncharacterized humoral factor
  - Successful renal transplantation may decrease size of cysts
- Increased proliferation of cyst-lining epithelium
Unclear role of calcium oxalate crystals
Not necessarily secondary to dialysis
- Cyst formation occurs in some patients even before dialysis therapy

CLINICAL ISSUES

Epidemiology

Incidence
- 60-80% of patients on dialysis for more than 4 years may be diagnosed with ACD
Age
- All ages
Gender
- Male predilection
Ethnicity
- Africans may be more likely than Caucasians to develop ACD