Clear cell papillary renal cell tumour

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Clear cell papillary renal cell tumour
Diagnosis in short

Clear cell papillary renal cell tumour. H&E stain.
Synonyms previously known as clear cell tubulopapillary renal cell carcinoma and clear cell papillary renal cell carcinoma
LM low (ISUP nucleolar) grade apical nuclei, clear cells, pseudopapillae, cystic and/or tubular architectures
LM DDx clear cell renal cell carcinoma, papillary renal cell carcinoma, Xp11.2 translocation carcinoma
IHC CK7 +ve, AMACR -ve, CD10 -ve, TFE3 -ve
Grossing notes total nephrectomy for tumour grossing, partial nephrectomy grossing
Staging kidney cancer staging
Site kidney - see renal tumours
Prevalence uncommon
Prognosis good
Clin. DDx other renal tumours

Clear cell papillary renal cell tumour, abbreviated CCPRCT, is a relatively common benign renal tumour.

It was previously known as clear cell papillary renal cell carcinoma, abbreviated CCPRCC

It is also previously known as clear cell tubulopapillary renal cell carcinoma,[1] abbreviated CCTPRCC.

General

Microscopic

Features:[1]

  • Some features of both:
    1. Clear cell RCC.
      • Clear cells.
    2. Papillary RCC.
      • Pseudopapillae - often do not have true papillae.
  • Apical nuclei - should be predominant ‡ - key feature.[8]
    • In most glandular structures the nuclei are usu. basally located, i.e. in the cytoplasm adjacent to the basement membrane.
  • Tubular and/or cystic architecture.

Notes: ‡

DDx:

Note:

  • Renal angiomyoadenomatous tumour has been proposed as separate entity.[9] The Vancouver modification of the WHO classification does not consider it a separate entity; it lumps it with clear cell papillary renal cell carcinoma.

Images

  • CCPRCT - high mag. (WC/Nephron)

  • CCPRCT - very high mag. (WC/Nephron)

www:

IHC

Features:[1]

Others:[1]

How to remember: The two key stains are opposite of what the name implies.

  • Clear cell RCC: CK7 negative. In this tumour it is the opposite - CK7 is positive.
  • Papillary RCC: AMACR positive. In this tumour it is the opposite - AMACR is negative.

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Left Kidney Mass, Core Biopsy:
     - CLEAR CELL PAPILARY RENAL CELL TUMOUR, preliminary ISUP nucleolar grade 1 of 4, see comment.

Comment:
The tumour has a tubulocystic architecture and is composed of cuboidal cells with clear cytoplasm. The nuclei are reverse stratified.

The tumour stains as follows: 
POSITIVE: PAX8 (diffuse), CK7 (strong, diffuse), CK34betaE12 (strong, diffuse), vimentin (diffuse, strong), EMA (moderate, diffuse).
NEGATIVE: CD10.

The findings are in keeping with clear cell tubulopapillary renal cell tumour. These tumours were previously called "clear cell papillary renal cell carcinoma".


These tumours have a highly favourable clinical behaviour.[1,2] 

1. Am J Surg Pathol. 2015 Dec;39(12):1621-34. DOI: 10.1097/PAS.0000000000000513
2. Pathology. 2020 Nov 19;S0031-3025(20)30957-0. DOI: 10.1016/j.pathol.2020.10.002

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Rohan, SM.; Xiao, Y.; Liang, Y.; Dudas, ME.; Al-Ahmadie, HA.; Fine, SW.; Gopalan, A.; Reuter, VE. et al. (Sep 2011). "Clear-cell papillary renal cell carcinoma: molecular and immunohistochemical analysis with emphasis on the von Hippel-Lindau gene and hypoxia-inducible factor pathway-related proteins.". Mod Pathol 24 (9): 1207-20. doi:10.1038/modpathol.2011.80. PMID 21602815.
  2. ↑ Srigley, JR.; Delahunt, B.; Eble, JN.; Egevad, L.; Epstein, JI.; Grignon, D.; Hes, O.; Moch, H. et al. (Oct 2013). "The International Society of Urological Pathology (ISUP) Vancouver Classification of Renal Neoplasia.". Am J Surg Pathol 37 (10): 1469-89. doi:10.1097/PAS.0b013e318299f2d1. PMID 24025519.
  3. ↑ Rohan, SM.; Xiao, Y.; Liang, Y.; Dudas, ME.; Al-Ahmadie, HA.; Fine, SW.; Gopalan, A.; Reuter, VE. et al. (Sep 2011). "Clear-cell papillary renal cell carcinoma: molecular and immunohistochemical analysis with emphasis on the von Hippel-Lindau gene and hypoxia-inducible factor pathway-related proteins.". Mod Pathol 24 (9): 1207-20. doi:10.1038/modpathol.2011.80. PMID 21602815.
  4. ↑ Aydin, H.; Chen, L.; Cheng, L.; Vaziri, S.; He, H.; Ganapathi, R.; Delahunt, B.; Magi-Galluzzi, C. et al. (Nov 2010). "Clear cell tubulopapillary renal cell carcinoma: a study of 36 distinctive low-grade epithelial tumors of the kidney.". Am J Surg Pathol 34 (11): 1608-21. doi:10.1097/PAS.0b013e3181f2ee0b. PMID 20924276.
  5. ↑ Diolombi, ML.; Cheng, L.; Argani, P.; Epstein, JI. (Dec 2015). "Do Clear Cell Papillary Renal Cell Carcinomas Have Malignant Potential?". Am J Surg Pathol 39 (12): 1621-34. doi:10.1097/PAS.0000000000000513. PMID 26426379.
  6. ↑ Williamson SR (January 2021). "Clear cell papillary renal cell carcinoma: an update after 15 years". Pathology 53 (1): 109–119. doi:10.1016/j.pathol.2020.10.002. PMID 33223139.
  7. ↑ Zhou, H.; Zheng, S.; Truong, LD.; Ro, JY.; Ayala, AG.; Shen, SS. (Oct 2013). "Clear cell papillary renal cell carcinoma is the fourth most common histologic type of renal cell carcinoma in 290 consecutive nephrectomies for renal cell carcinoma.". Hum Pathol. doi:10.1016/j.humpath.2013.08.004. PMID 24182559.
  8. ↑ Bing, Z.; Tomaszewski, JE. (2011). "Case Report: Clear Cell Papillary Renal Cell Carcinoma in the Bilateral Native Kidneys after 2 Years of Renal Transplantation: Report of a Case and Review of the Literature.". Case Reports in Transplantation 2011. doi:10.1155/2011/387645. http://www.hindawi.com/crim/transplantation/2011/387645/cta/.
  9. ↑ Petersson, F.; Grossmann, P.; Hora, M.; Sperga, M.; Montiel, DP.; Martinek, P.; Gutierrez, ME.; Bulimbasic, S. et al. (Jul 2013). "Renal cell carcinoma with areas mimicking renal angiomyoadenomatous tumor/clear cell papillary renal cell carcinoma.". Hum Pathol 44 (7): 1412-20. doi:10.1016/j.humpath.2012.11.019. PMID 23434146.
  10. ↑ Williamson, SR.; Gupta, NS.; Eble, JN.; Rogers, CG.; Michalowski, S.; Zhang, S.; Wang, M.; Grignon, DJ. et al. (Nov 2015). "Clear Cell Renal Cell Carcinoma With Borderline Features of Clear Cell Papillary Renal Cell Carcinoma: Combined Morphologic, Immunohistochemical, and Cytogenetic Analysis.". Am J Surg Pathol 39 (11): 1502-10. doi:10.1097/PAS.0000000000000514. PMID 26457355.
  11. ↑ He, H.; Zhou, GX.; Zhou, M.; Chen, L. (Sep 2011). "The distinction of clear cell carcinoma of the female genital tract, clear cell renal cell carcinoma, and translocation-associated renal cell carcinoma: an immunohistochemical study using tissue microarray.". Int J Gynecol Pathol 30 (5): 425-30. doi:10.1097/PGP.0b013e318214dd4f. PMID 21804394.
  12. ↑ Pramick, M.; Ziober, A.; Bing, Z. (Oct 2013). "Useful immunohistochemical panel for differentiating clear cell papillary renal cell carcinoma from its mimics.". Ann Diagn Pathol 17 (5): 437-40. doi:10.1016/j.anndiagpath.2013.05.004. PMID 23791764.
  13. ↑ Williamson, SR.; Eble, JN.; Cheng, L.; Grignon, DJ. (May 2013). "Clear cell papillary renal cell carcinoma: differential diagnosis and extended immunohistochemical profile.". Mod Pathol 26 (5): 697-708. doi:10.1038/modpathol.2012.204. PMID 23238627.
  14. ↑ Kuroda, N.; Ohe, C.; Kawakami, F.; Mikami, S.; Furuya, M.; Matsuura, K.; Moriyama, M.; Nagashima, Y. et al. (2014). "Clear cell papillary renal cell carcinoma: a review.". Int J Clin Exp Pathol 7 (11): 7312-8. PMID 25550767.
  15. ↑ Hakimi, AA.; Tickoo, SK.; Jacobsen, A.; Sarungbam, J.; Sfakianos, JP.; Sato, Y.; Morikawa, T.; Kume, H. et al. (Jun 2015). "TCEB1-mutated renal cell carcinoma: a distinct genomic and morphological subtype.". Mod Pathol 28 (6): 845-53. doi:10.1038/modpathol.2015.6. PMID 25676555.
  16. ↑ Mantilla JG, Antic T, Tretiakova M (August 2017). "GATA3 as a valuable marker to distinguish clear cell papillary renal cell carcinomas from morphologic mimics". Hum Pathol 66: 152–158. doi:10.1016/j.humpath.2017.06.016. PMID 28705707.
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