Kidney tumor

Adult renal cell carcinoma - rare

Papillary renal neoplasm with reverse polarity


Editorial Board Member: Debra L. Zynger, M.D.
Deputy Editor-in-Chief: Maria Tretiakova, M.D., Ph.D.
Hsin-Yi Chang, M.D.
Jen-Fan Hang, M.D.

Topic Completed: 8 November 2021

Minor changes: 8 November 2021

Copyright: 2021, PathologyOutlines.com, Inc.

PubMed Search: Papillary renal neoplasm with reverse polarity

Hsin-Yi Chang, M.D.
Jen-Fan Hang, M.D.
Page views in 2021 to date: 754
Cite this page: Chang HY, Hang JF. Papillary renal neoplasm with reverse polarity. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumorPRNRP.html. Accessed December 3rd, 2021.
Definition / general
  • Clinically indolent papillary renal cell tumor characterized by oncocytic granular tumor cells in papillary or tubulopapillary architecture, apically located nuclei, diffuse and strong GATA3 expression and frequent KRAS mutation
Essential features
  • Predominant thin papillary or tubulopapillary growth
  • Tumor cells with eosinophilic, finely granular cytoplasm and low grade, apically located nuclei
  • Diffuse and strong GATA3 nuclear staining
  • Recurrent KRAS missense mutation at codon 12
  • Indolent, no recurrence or metastasis reported
Terminology
ICD coding
  • ICD-O:
    • 8260/0 - papillary adenoma
    • 8260/3 - papillary renal cell carcinoma
Epidemiology
Sites
  • Kidney, renal cortex
Pathophysiology
Etiology
  • No specific risk factors reported
Clinical features
Diagnosis
Radiology description
  • MRI: hyperintense in T1 and hypointense in T2
  • Ultrasonography: isoechoic or heterogeneous mass with a sharp margin
Radiology images

Contributed by Hsin-Yi Chang, M.D. and Jen-Fan Hang, M.D.
Hyperintense in T1 weighted MRI

Hyperintense in T1 weighted MRI

Hypointense in T2 weighted MRI

Hypointense in T2 weighted MRI

Isoechoic mass under ultrasound

Isoechoic mass under ultrasound

Case reports
Gross description
Gross images

Images hosted on other servers:

Courtesy of Katrina Collins, M.D.
PRNRP in polycystic kidney disease

In polycystic kidney disease

PRNRP with delicate papillae

Delicate papillae

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Hsin-Yi Chang, M.D. and Jen-Fan Hang, M.D.
Partially cystic growth

Partially cystic growth

Papillary pattern

Papillary pattern

Tubulopapillary pattern

Tubulopapillary pattern

Stromal hyalinization

Stromal hyalinization


Low grade inverted nuclei

Low grade inverted nuclei

Type D papillary adenoma

Type D papillary adenoma

GATA3 staining

GATA3

Molecular / cytogenetics description
Sample pathology report
  • Kidney, right, partial nephrectomy:
    • Papillary renal neoplasm with reverse polarity (see comment)
    • Comment: Papillary renal neoplasm with reverse polarity is currently considered to be a histologic variant of papillary renal cell carcinoma; however, recent studies suggest that it has a very indolent clinical behavior. No recurrence or metastasis has been reported.
Differential diagnosis
Board review style question #1

Which of the following is the most useful immunohistochemical marker to differentiate papillary renal neoplasm with reverse polarity from other renal cell carcinomas with eosinophilic cytoplasm?

  1. CD10
  2. CD117
  3. CK7
  4. GATA3
Board review style answer #1
D. GATA3. In renal cell carcinomas (RCCs) with eosinophilic cytoplasm, GATA3 positivity is only seen in papillary renal neoplasm with reverse polarity (PRNRP). Interestingly, GATA3 positivity is reported in another indolent neoplasm with reverse polarity but optically clear cytoplasm, namely clear cell papillary RCC. CK7 is expressed in PRNRP but also in type 1 papillary RCC and chromophobe RCC. Though CD117 is positive in chromophobe RCC and oncocytoma, positive weak staining for PRNRP is also reported in a small percentage of cases. CD10 shows variable expression in papillary RCC, chromophobe RCC, oncocytoma and PRNRP (Hum Pathol 2017;66:152, Mod Pathol 2005;18:535, Pathol Int 2009;59:137, Mod Pathol 2020;33:690, Int J Surg Pathol 2020;28:728, Am J Surg Pathol 2021 Aug 5 [Epub ahead of print]).

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Reference: Papillary renal neoplasm with reverse polarity
Board review style question #2

Which of the following genes is commonly mutated in papillary renal neoplasm with reverse polarity?

  1. FH
  2. KRAS
  3. MET
  4. VHL
Board review style answer #2
B. KRAS. Papillary renal neoplasm with reverse polarity is characterized by KRAS hotspot mutation at codon 12. Loss of function mutation of VHL gene is the molecular hallmark of clear cell renal cell carcinoma (RCC). MET alterations are frequently found in papillary RCC. FH mutations are diagnostic for FH deficient RCC.

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Reference: Papillary renal neoplasm with reverse polarity
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