Kidney tumor

Adult renal cell carcinoma - common

Papillary


Deputy Editor-in-Chief: Debra L. Zynger, M.D.
Nicole K. Andeen, M.D.
Maria Tretiakova, M.D., Ph.D.

Last author update: 1 March 2018
Last staff update: 26 May 2023 (update in progress)

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PubMed Search: "Papillary type" renal cell carcinoma kidney

See Also: Type 1, Type 2, Solid pattern

Nicole K. Andeen, M.D.
Maria Tretiakova, M.D., Ph.D.
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Cite this page: Andeen NK, Tretiakova M. Papillary. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumormalignantrccpap.html. Accessed June 9th, 2023.
Definition / general
Essential features
  • Papillary renal cell carcinoma (PRCC) comprises more than one entity which may have morphologic overlap but have distinct molecular pathways and clinical behavior, currently grouped at least into type 1 and type 2 PRCC
  • Subclassification (type 1 or 2) and exclusion of other renal cell carcinomas with papillary architecture is important
    • Type 1 PRCC:
      • Papillae are lined by a single layer of cells with scanty basophilic cytoplasm and low nuclear grade
      • Have +7, +17 and other trisomies, MET mutations, better prognosis
    • Type 2 PRCC:
      • Papillae are lined by pseudostratified layers of cells with more abundant eosinophilic cytoplasm and higher nucleolar grade
      • Multiple genetic changes described, likely represents more than one entity, worse prognosis
Epidemiology
Sites
  • Kidney
Pathophysiology
  • Depends on subtype
Etiology
Clinical features
Diagnosis
  • In one series of papillary renal cell carcinomas, 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 images

Images hosted on other servers:

CT scan upper pole tumor

Prognostic factors
  • Need to distinguish between type 1 and type 2 papillary renal cell carcinoma for accurate prognosis
Case reports
Treatment
Gross description
Gross images

Contributed by Nicole K. Andeen, M.D. and Maria Tretiakova, M.D., Ph.D.

PRCC



Images hosted on other servers:

Tan pink mass with satellite nodules

Renal vein extension

Large cystic tumor infiltrates perirenal fat

Hemorrhagic and partially necrotic tumor

Multiple tumor nodules, one is hemorrhagic cystic

Capsular invasion

Frozen section images

Contributed by Debra L. Zynger, M.D.

Delicate papillary cores

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Nicole K. Andeen, M.D., Maria Tretiakova, M.D., Ph.D., Semir Vranić, M.D., Ph.D. and @SueEPig on Twitter

Type 1

Type 2

Mixed type 1 and type 2


Type 1 PRCC

Type 2 PRCC with focal CK7 expression and diffuse AMACR expression (red)


Oncocytic variant of papillary renal cell carcinoma

Papillary renal cell carcinoma Papillary renal cell carcinoma

Papillary renal cell carcinoma

Cytology description
Cytology images

Contributed by Debra L. Zynger, M.D.

Papillary structures and foamy histiocytes



Images hosted on other servers:

Foamy macrophages

Negative stains
IHC panels
 Hale  KIT  CK7  S100A1  VIM  CAIX  AMACR  SDH  TFE3
 Chromophobe RCC   +++   +++   +++   -   -   -   -   +++   - 
 Clear cell RCC   -   -   -   -   +++   +++   -   +++   - 
 Oncocytoma   -   +++   rare   +++   -   -   -   +++   - 
 Papillary RCC   -   -   +++   -   +++   -   +++   +++   - 
 Translocation RCC   -   -   -   -   -   -   ++   +++   +++ 
 SDH deficient RCC   -   -   -   -   -   -   -   -   - 

References: Pathol Res Pract 2015;211:303, Ann Diagn Pathol 2020;44:151448, Am J Surg Pathol 2014;38:e6, Arch Pathol Lab Med 2019;143:1455, Transl Androl Urol 2019;8:S123, Hum Pathol 2020 Jul 13 [Epub ahead of print]
Electron microscopy description
  • Variably sized microvilli, small amounts of cytoplasmic lipid, no glycogen
Molecular / cytogenetics description
Differential diagnosis
Board review style question #1
Given the sometimes broad differential, what is generally the most useful group of stains for evaluating a renal cell carcinoma with papillary architecture?

  1. CAIX, CK7, AMACR, TFE3
  2. p53, p63, CD10
  3. PAX8, RCC, Hale colloidal iron
  4. Vimentin, RCC, CD10
Board review style answer #1
A. CAIX, CK7, AMACR, TFE3

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