Kidney tumor
Adult renal cell carcinoma - common
Papillary type 2
Last author update: 1 December 2017
Last staff update: 20 December 2023 (update in progress)
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PubMed Search: "Type 2" papillary renal cell carcinoma (eosinophilic)
See Also: Papillary type, renal cell carcinoma, type 1, solid growth pattern
Table of Contents
Definition / general | Essential features | Sites | Clinical features | Diagnosis | Radiology description | Prognostic factors | Case reports | Treatment | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosis | Board review style question #1 | Board review style answer #1Cite this page: Andeen NK, Tretiakova M. Papillary type 2. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumormalignantrccpaptyp2.html. Accessed April 26th, 2024.
Definition / general
- Papillary architecture with pseudostratified layers of cells with abundant eosinophilic cytoplasm and atypical nuclei with prominent nucleoli
- Poorer prognosis than type 1; typically presents at higher stage
- Type 1 vs. type 2 described in 1997 by Delahunt and Elbe (Mod Pathol 1997;10:537)
Essential features
- Papillary architecture with pseudostratified cells, abundant eosinophilic to occasionally clear cytoplasm, atypical nuclei, prominent nucleoli (usually ISUP grade 3 or higher)
- As currently defined, likely encompasses more than one entity (Am J Surg Pathol 2014;38:e35)
Sites
- Kidney
Clinical features
- More locally advanced disease and higher rates of metastasis than type 1 PRCC (Am J Surg Pathol 2014;38:887)
Diagnosis
- In one series of papillary RCCs, roughly 25% had classical type 1 features, 25% were type 2 and 50% had some degree of overlapping features (Am J Surg Pathol 2014;38:887)
Radiology description
- Radiology techniques may detect differences between type 1 and type 2 PRCC (Abdom Radiol (NY) 2017;42:1911)
Prognostic factors
- Stage for stage, worse prognosis and survival times than type 1 PRCC (Am J Surg Pathol 2014;38:887)
- Outcome is more comparable to clear cell RCC than to type 1 PRCC (PLoS One 2017;12:e0184173)
- Presents at a more advanced stage than clear cell RCC (Eur J Surg Oncol 2016;42:1744)
Case reports
- 52 year old woman with type 2 papillary renal cell carcinoma resistant to sunitinib who responded to axitinib (Nihon Hinyokika Gakkai Zasshi 2014;105:129)
- 64 year old woman with splenic metastasis (Hinyokika Kiyo 2017;63:371)
- 66 year old man with tumor demonstrating cavitary necrosis (Mol Clin Oncol 2013;1:318)
Treatment
- Resection
- Tyrosine kinase inhibitors including sunitinib (Ann Oncol 2015;26:1123)
- Systemic therapy varies (J Natl Compr Canc Netw 2017;15:804, NCCN: NCCN guidelines version 2.2017 [Accessed: 30 November 2017])
Gross images
Images hosted on other servers:
Irregular friable yellow tumor of the upper pole
Large cystic tumor
Microscopic (histologic) description
- Papillary architecture (Am J Surg Pathol 2014;38:887) with:
- Pseudostratified layers of large cells (most important feature)
- Abundant eosinophilic cytoplasm
- Atypical nuclei with prominent nucleoli (usually ISUP grade 3 or higher)
- May have areas of clear cytoplasm
- Variable necrosis
- Less frequent foam cells and psammoma bodies than type 1
- Higher nuclear grade than type 1
- Grading based on ISUP nucleolar grade
Microscopic (histologic) images
Contributed by Nicole K. Andeen, M.D. and Maria Tretiakova, M.D., Ph.D.
Type 2 PRCC with focal CK7 expression and diffuse AMACR expression (red)
Images hosted on other servers:
Fig. B
Cytology description
- Papillary structures, foamy histiocytes, intracytoplasmic hemosiderin and nuclear grooves
Positive stains
Negative stains
Electron microscopy description
- Similar to classic type but with more mitochondria
Molecular / cytogenetics description
- Type 1 pRCC has more consistent chromosomal aberrations than type 2, including:
- Heterogeneous, losses or gains of chromosomes 1, 3, 4, 5, 6, 8, 9, 10, 11, 15, 18 and 22 (Cancer Genet Cytogenet 2010;199:128, J Clin Oncol 2009;27:746, Am J Pathol 2002;161:997, Am J Surg Pathol 2017;41:1618)
- Enriched in NRF-ARE2 pathway (Am J Surg Pathol 2017;41:1618)
- 8q gains and allelic imbalance of 9q13 have prognostic significance (J Pathol 2000;190:457)
- Molecular signatures are different between PRCC type 1 and 2 (Cancer Res 2005;65:5628), and potentially a third type of PRCC (Am J Surg Pathol 2017;41:1618)
- In advanced stage type 2 PRCC, commonly altered genes are CDKN2A / B (18%), TERT (18%), NF2 (13%) and FH (13%) (Eur Urol 2017 May 30 [Epub ahead of print])
Differential diagnosis
- Acquired cystic disease associated RCC (ACD-RCC): occurs in setting of end stage renal disease, often has sieve-like architecture and abundant calcium oxalate crystals
- Clear cell RCC with cytoplasmic eosinophilia: clear cell RCC may have papillary component but is CAIX positive and AMACR negative
- Clear cell tubulopapillary RCC: is lower nuclear grade and diffusely CK7 positive with cup-like CAIX staining
- HLRCC: may have many similar features but some cells have distinct, prominent eosinophilic nucleolus with clear halo (CMV-like) and has biallelic inactivation of fumarate hydratase
- MiTF associated RCC: may also be papillary with eosinophilic and clear cells; express cathepsin K and some express HMB45 or MelanA; FISH for TFE3 tends to be technically superior to IHC
- Type 1 PRCC: type 2 PRCC has more cytologic pleomorphism, pseudostratification and cells are more eosinophilic; foamy macrophages and psammoma bodies are less common; type 2 PRCC has patchy weak or negative CK7 and EMA and higher expression of Ki67 and p53 than type 1 PRCC (Am J Surg Pathol 2014;38:887)
Board review style question #1
Type 2 papillary renal cell carcinoma:
- Has a consistently better clinical outcome than clear cell RCC
- Has a diagnostic recurrent genetic abnormality
- Is heterogeneous and as currently defined likely encompasses more than one entity
- Usually forms a single layer of cells on papillae and is low nuclear grade
Board review style answer #1
C. Is heterogeneous and as currently defined likely encompasses more than one entity
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Reference: Type 2 (eosinophilic) papillary renal cell carcinoma
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Reference: Type 2 (eosinophilic) papillary renal cell carcinoma
