Clear cell papillary renal cell tumour

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Clear cell papillary renal cell tumour
Diagnosis in short

Clear cell papillary renal cell carcinoma. H&E stain.
Synonyms clear cell tubulopapillary renal cell carcinoma, clear cell papillary cystadenoma (proposed name), clear cell papillary neoplasm of low malignant potential (proposed name)
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 carcinoma, abbreviated CCPRCC, is recently described type of renal cell carcinoma.

It is also 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

  • CCPRCC - high mag. (WC/Nephron)

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

www:

IHC

Features:[1]

Others:[1]

  • HIF-1alpha +ve.
  • GLUT-1 +ve.
  • CA9 +ve (cup-shaped pattern).[14]
  • CK34betaE12 +ve.[15]

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 TUBULOPAPILARY RENAL CELL CARCINOMA (clear cell 
       papillary renal cell carcinoma), 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 carcinoma. 

These tumours have a highly favourable clinical behaviour.[1,2] The terms "clear cell papillary neoplasm of low malignant potential"[1] and "clear cell papillary (cyst)adenoma"[2] have been proposed as names that better reflect their biology.

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.
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