Localized cystic disease of the kidney
|Localized cystic disease of the kidney|
|Diagnosis in short|
cysts with cuboidal or flat lining epithelium - contiguous with renal collecting ducts;renal parenchyma (renal tubules or glomeruli) with in septations; +/-foamy histiocytes; +/-refractile material within the cysts
|LM DDx||cystic renal tumours (cystic clear cell renal cell carcinoma, cystic nephroma, multilocular cystic renal neoplasm of low malignant potential), hereditary cystic renal disease (autosomal dominant polycystic kidney disease)|
|Signs||+/-hematuria (uncommon), often incidentaloma|
|Symptoms||abdominal pain - due to mass effect|
|Radiology||localized cystic disease, Bosniak classification 2 or 3|
|Clin. DDx||cystic renal cell carcinoma|
|Treatment||excision for diagnosis|
- Rare and benign.
- No family history of polycystic kidney disease.
- Usually an incidental finding (7 of 9 cases).
- Hematuria - uncommon (2 of 9 cases).
- Focal cystic lesion(s) without a capsule involving the renal papillae +/-renal cortex.
- Contain clear or light-yellow serous fluid.
- Focal - only part of the kidney should be involved.
- Cysts with cuboidal or flat lining epithelium.
- Contiguous with renal collecting ducts.
- Renal parenchyma (renal tubules or glomeruli) with in (cyst) septations.
- +/-Foamy histiocytes.
- +/-Refractile material within the cysts.
Notes - negatives:
- No significant inflammation.
- No capsule.
- No papillary hyperplasia.
- Cystic renal tumours.
- Hereditary cystic renal disease, e.g. autosomal dominant polycystic kidney disease.
Left Kidney, Radical Nephrectomy: - Localized cystic disease of the kidney, see microscopic description. - NEGATIVE for malignancy.
The sections show multiple large cortical cysts. There is relative sparing of the parenchyma in the lower pole. The cyst walls are fibrotic, contain parenchymal remnants and have foci of calcifications. The cyst lining consists of flatted epithelium without appreciable cytoplasm. No cyst lining cell atypia is present. The cyst lining cells are POSITIVE for AE1/AE3 and NEGATIVE for CD34.
Comment: It is noted that the contralateral kidney is unremarkable on imaging. The findings are in keeping with localized cystic disease of the kidney, a rare benign finding that can mimic a tumour.
1. Am J Surg Pathol 37 (4): 506-13. doi:10.1097/PAS.0b013e318271eff9. PMID: 23211292.
- Ding, Y.; Chen, L.; Deng, FM.; Melamed, J.; Fan, R.; Bonsib, S.; Zhou, M. (Apr 2013). "Localized cystic disease of the kidney: distinction from cystic neoplasms and hereditary polycystic diseases.". Am J Surg Pathol 37 (4): 506-13. doi:10.1097/PAS.0b013e318271eff9. PMID 23211292.
- Bhat, S.; Thomas, A. (Oct 2007). "Benign multilocular cyst or multilocular cystic nephroma in adults: three case reports.". Indian J Surg 69 (5): 209-11. doi:10.1007/s12262-007-0025-1. PMID 23132986.