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.
LM apical nuclei, clear cells, pseudopapillae, cystic and/or tubular architectures
LM DDx papillary renal cell carcinoma, clear cell renal cell carcinoma, Xp11.2 translocation carcinoma
IHC CK7 +ve, AMACR -ve, CD10 -ve, TFE3 -ve
Site kidney - see renal tumours
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]

General

  • New entity not in the WHO classification.
  • Shown to be distinct on a molecular basis.[2]

Microscopic

Features:[1]

  • Features of both:
    1. Clear cell RCC.
      • Clear cells.
    2. Papillary RCC.
      • Pseudopapillae - often do not have true papillae.
  • Apical nuclei - key feature.[3]
    • 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.

DDx:

Images

  • CCPRCC - high mag. (WC/Nephron)

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

www:

IHC

Features:[1]

  • CK7 +ve.
    • Usually -ve in clear cell RCC.
  • CD10 -ve.
    • Usually +ve in clear cell RCC.
    • Positive in Xp11.2 translocation carcinoma.[4]
  • TFE3 -ve.
  • AMACR -ve.
  • Smooth muscle actin +ve (focal).[5]

Others:[1]

  • HIF-1alpha +ve.
  • GLUT-1 +ve.

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.

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 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.
  2. ↑ 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.
  3. ↑ 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/.
  4. ↑ 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.
  5. ↑ 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.
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