Acquired cystic disease subtype renal cell carcinoma (ACD-RCC): prevalence and imaging features at a single institution

Abdom Radiol (NY). 2022 Aug;47(8):2858-2866. doi: 10.1007/s00261-022-03566-6. Epub 2022 Jun 8.


Purpose: Acquired cystic kidney disease (ACKD) is commonly seen in patients with end-stage renal disease (ESRD), and patients with ACKD have an increased risk of renal cell carcinoma (RCC). Acquired cystic disease-associated RCC (ACD-RCC) was incorporated into the 2016 World Health Organization Classification. This study aims to describe the imaging features of ACD-RCC, which are not well reported previously.

Methods: Retrospective review of patients with ACKD who underwent total nephrectomy for concern of a renal mass between 2016 and 2021 yielded 122 nephrectomies in 107 patients. Pathology reports were searched for type and subtype of mass. In ACD-RCC subtypes, imaging studies were evaluated for modality and contrast enhancement (CE). Imaging findings assessed included cystic/solid nature, unenhanced CT (NECT) attenuation, enhancement characteristics [non-enhancing (< 10 HU difference), equivocal (10-20 HU), enhancing (> 20 HU)], subjective MRI enhancement, T1 and T2 signal intensity, restricted diffusion, ultrasound (US) echogenicity, and subjective CEUS enhancement.

Results: 148 masses were identified, 122 (82%) of which were malignant and 26 (18%) benign. The three most common tumors were clear cell RCC (n = 47), papillary RCC (n = 35), and ACD-RCC (n = 21). Of the 21 cases of ACD-RCC, 16 had preoperative imaging: CT (15: 6 NECT only, 2 CECT only, 7 combined NECT and CECT), MRI (4), CEUS (5). Ten of these tumors were solid/mostly solid and 6 mixed cystic/solid. On NECT, the average attenuation was 35 HU (range 13-52). Of those with multiphasic CTs, 1 was non-enhancing, 3 were equivocal, and 3 enhanced. All 3 masses imaged with CE-MRI showed enhancement. All 4 tumors evaluated by MRI demonstrated T2 hypointensity and restricted diffusion. All five masses enhanced on CEUS.

Conclusion: ACD-RCC subtype was the third most common renal neoplasm in ACKD patients. Our findings found that no single imaging feature is pathognomonic for ACD-RCC. However, ACD-RCCs are typically solid masses with most demonstrating equivocal or mild enhancement on CT. T2 hypointensity and restricted diffusion were the most common MRI features.

Keywords: Acquire cystic kidney disease; Acquired cystic disease-associated renal cell carcinoma; Renal cell carcinoma End-stage renal disease.

MeSH terms

  • Carcinoma, Renal Cell* / diagnostic imaging
  • Carcinoma, Renal Cell* / pathology
  • Humans
  • Kidney Failure, Chronic* / pathology
  • Kidney Failure, Chronic* / surgery
  • Kidney Neoplasms* / diagnostic imaging
  • Kidney Neoplasms* / pathology
  • Nephrectomy
  • Prevalence
  • Retrospective Studies