Kidney tumor
Adult renal cell carcinoma
Acquired cystic disease associated renal cell carcinoma

Author: Nicole K. Andeen, M.D. (see Authors page)
Editor: Maria Tretiakova, M.D., Ph.D.

Revised: 28 August 2017, last major update August 2017

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: "Acquired cystic disease" associated renal cell carcinoma

Cite this page: Andeen, N. K. Acquired cystic disease associated renal cell carcinoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/kidneytumormalignantacquiredcysticRCC.html. Accessed November 24th, 2017.
Definition / general
Essential features
Epidemiology
  • ACD occurs in 35% of long term dialysis patients; of these, 6% develop RCC
Sites
  • Kidney
Clinical features
Case reports
Gross description
Microscopic (histologic) description
  • Cribriform / microcystic / sieve-like architecture
  • Abundant granular eosinophilic cytoplasm with prominent nucleoli
  • Intratumoral calcium oxalate crystals are very common but not necessary for diagnosis (Am J Surg Pathol 2005;29:443)
  • May be nodules arising from cyst walls or masses separated from cysts (Am J Surg Pathol 2013;37:1469)
  • Sometimes prominent clear cell cytology
Microscopic (histologic) images

Images hosted on PathOut server:

Images contributed by Nicole Andeen, M.D. and Maria Tretiakova, M.D., Ph.D.

ACD RCC with papillary architecture, eosinophilic cells and abundant calcium oxalate



Images hosted on other servers:

Calcium oxalate crystals

Various images

Cytology description
  • Moderately cellular, papillary clusters of polygonal to columnar cells with abundant eosinophilic granular cytoplasm, round and central nuclei, finely granular chromatin, prominent central grade 3 nucleoli (Diagn Cytopathol 2008;36:344)
Negative stains
Molecular / cytogenetics description
Differential diagnosis
  • Clear cell renal cell carcinoma: both entities may have clear cells and tubular architecture
    • ACD RCC has regions of eosinophilic cells, papillary architecture, oxalate crystals and background cystic renal parenchyma
  • Papillary renal cell carcinoma: both entities may have papillary architecture and eosinophilic cells
    • ACD RCC has characteristic sieve-like architecture, oxalate crystals and background cystic renal parenchyma
    • CK7 is usually negative in ACD RCC but positive in PRCC
Board review question #1
What are the most helpful features to distinguish acquired cystic disease associated renal cell carcinoma from other RCCs?

  1. Intratumoral oxalate crystals and background of ESRD with cysts
  2. Presence of papillary architecture and eosinophilic cells
  3. Specific recurrent cytogenetic abnormality
  4. Unique immunohistochemical profile
Board review answer #1
A. Intratumoral oxalate crystals and background of ESRD with cysts