OSU Department of Pathology

Anatomical Pathology Interesting Case Conference

Microscopic Description:

Histologic examination revealed two distinct morphologic components:

  • Image 1A,1B,1C: Microscopic examination revealed a neoplasm exhibiting a branched papillary architecture with fibrovascular cores, lined by neoplastic epithelial cells showing nuclear grade 2 features, consistent with papillary renal cell carcinoma.
  • Image 2A : Consisted of cystic spaces lined by flattened cuboidal epithelium. The cyst lumina contained few oxalate crystals (IMAGE 2B,2C), and showed no evidence of malignancy.
  • Immunohistochemistry was positive for AE 1/3, PAX-8 and negative for CK-7.
Discussion:

Occurs predominantly in patients with end-stage renal disease (ESRD) receiving long-term hemodialysis or peritoneal dialysis, and may also develop in individuals with longstanding uremia.

Grossly, Kidneys are typically moderately enlarged with cortical and medullary cysts containing clear serous fluid. Tumors are frequently multifocal (50%) and bilateral (25%).

Microscopically, cysts lined by flattened or cuboidal epithelium that may show focal pseudo papillae with nuclear enlargement and loss of polarity. Complications include occasional hemorrhage into cysts and development of renal cell carcinoma. Cyst lumina may contain calcium oxalate crystals. The surrounding renal parenchyma demonstrates chronic renal injury characterized by global glomerulosclerosis, tubular atrophy, and interstitial fibrosis.

CT imaging typically shows well-defined, rounded lesions of variable size which may be solid, cystic or mixed. They are often isodense on non-contrast CT and may exhibit calcifications.

Nephrectomy is the standard treatment for ACD-associated RCC. Regular surveillance is recommended for patients with longstanding dialysis, particularly those for 10 years or more, due to increased risk of renal malignancy.

References:

Kondo T, Sasa N, Yamada H, Takagi T, Iizuka J, Kobayashi H, Yoshida K, Fukuda H, Ishihara H, Tanabe K, Tsuzuki T. Acquired cystic disease-associated renal cell carcinoma is the most common subtype in long-term dialyzed patients: Central pathology results according to the 2016 WHO classification in a multi-institutional study. Pathol Int. 2018 Oct;68(10):543-549. doi: 10.1111/pin.12718. Epub 2018 Sep 6.

 

Griffiths, D.F.R., and G.M. Vujanic. “Cystic Lesions of the Kidney—Selected Topics.” Current Diagnostic Pathology, vol. 8, no. 2, Apr. 2002, pp. 94–101, https://doi.org/10.1054/cdip.2001.0106.

Ishikawa I, Saito Y, Asaka M, Tomosugi N, Yuri T, Watanabe M, Honda R. Twenty-year follow-up of acquired renal cystic disease. Clin Nephrol. 2003 Mar;59(3):153-9. doi: 10.5414/cnp59153.

Rioux-Leclercq NC, Epstein JI. Renal cell carcinoma with intratumoral calcium oxalate crystal deposition in patients with acquired cystic disease of the kidney. Arch Pathol Lab Med. 2003 Feb;127(2):E89-92. doi: 10.5858/2003-127-e89-RCCWIC.

Denton MD, Magee CC, Ovuworie C, Mauiyyedi S, Pascual M, Colvin RB, Cosimi AB, Tolkoff-Rubin N. Prevalence of renal cell carcinoma in patients with ESRD pre-transplantation: a pathologic analysis. Kidney Int. 2002 Jun;61(6):2201-9. doi: 10.1046/j.1523-1755.2002.00374.x.

 

CASE IMAGES