Kidney tumor
Adult renal cell carcinoma - rare
Acquired cystic disease associated
Last author update: 1 August 2017
Last staff update: 17 March 2023 (update in progress)
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PubMed Search: "Acquired cystic disease" associated renal cell carcinoma
Table of Contents
Definition / general | Essential features | Epidemiology | Sites | Clinical features | Case reports | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosis | Board review style question #1 | Board review style answer #1Cite this page: Andeen NK, Tretiakova M. Acquired cystic disease associated. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumormalignantacquiredcysticRCC.html. Accessed March 23rd, 2023.
Definition / general
- Unique morphology of renal cell carcinoma (RCC) arising in kidneys with acquired cystic disease (ACD) due to end stage renal disease (ESRD), characterized by microcystic / sieve-like, papillary and solid architecture, eosinophilic and clear cells and abundant calcium oxalate crystals (Am J Surg Pathol 2006;30:141)
- Most common RCC in patients with ACD, comprising 36% of all epithelial neoplasms arising in ESRD (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016, Am J Surg Pathol 2013;37:1469)
Essential features
- Most common RCC in patients with ACD (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
- Abundant oxalate crystals
- Morphology: mixed solid, microcystic, papillary architecture with eosinophilic and clear cells
- Generally indolent behavior, perhaps due to early detection
Epidemiology
- ACD occurs in 35% of long term dialysis patients; of these, 6% develop RCC
Sites
- Kidney
Clinical features
- Often indolent clinical behavior, likely in part because of early detection with periodic imaging of patients with ESRD (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
- Tumors with sarcomatoid, rhabdoid or sometimes typical features can metastasize (Am J Surg Pathol 2006;30:141, Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
Case reports
- Clear cell and papillary subtypes with abundant calcium oxalate crystals (Arch Pathol Lab Med 2003;127:E89)
- Sarcomatoid change (Histol Histopathol 2008;23:1327)
Gross description
- Mass lesion in ACD background; usually 3 cm or less
- Multifocal (~50%) and bilateral (~25%) (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
Gross images
Contributed by Maria Tretiakova, M.D., Ph.D.
Solid and cystic hemorrhagic neoplasm
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
Contributed by Nicole Andeen, M.D., Maria Tretiakova, M.D., Ph.D. and Gregory T. MacLennan, M.D.
Acquired cystic disease RCC
Oxalate crystals
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)
Positive stains
- Specific immunohistochemical profile is not required for diagnosis (Am J Surg Pathol 2013;37:1469)
- CD10, AE1 / AE3 (Mod Pathol 2006;19:780), AMACR (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
Negative stains
- EMA, CK7 (but may be focally positive; Am J Surg Pathol 2013;37:1469)
Molecular / cytogenetics description
- Comparative genomic microarray and FISH studies reveal gains and losses of multiple chromosomes (Am J Surg Pathol 2013;37:1469)
- Gains of sex chromosomes and gains of 3, 7, 16, 17 (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
- High prevalence of gains of Y, 3 and 16 distinguishes from papillary RCC, which also has gains in chromosomes 7 and 17 (Moch: WHO Classification of Tumours of the Urinary System and Male Genital Organs, 4th Edition, 2016)
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 style question #1
What are the most helpful features to distinguish acquired cystic disease associated renal cell carcinoma from other RCCs?
- Intratumoral oxalate crystals and background of ESRD with cysts
- Presence of papillary architecture and eosinophilic cells
- Specific recurrent cytogenetic abnormality
- Unique immunohistochemical profile
Board review style answer #1
A. Intratumoral oxalate crystals and background of ESRD with cysts
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