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 |
|
| |
| 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]
General
- New entity - not in the 2004 WHO classification of renal tumours.[1]
- Shown to be distinct on a molecular basis.[2]
- No vHL gene mutations,[3] seen in clear cell renal cell carcinoma.
- Good prognosis - based on a small series.[3]
- Fourth most common type of RCC is a recent series.[4]
- Most common (in order): clear cell RCC, papillary RCC, chromophobe RCC, clear cell papillary RCC.
Microscopic
Features:[1]
- Features of both:
- Clear cell RCC.
- Clear cells.
- Papillary RCC.
- Pseudopapillae - often do not have true papillae.
- Clear cell RCC.
- Apical nuclei - key feature.[5]
- 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.[6]
- TFE3 -ve.
- By definition +ve in Xp11.2 translocation carcinoma.
- AMACR -ve.
- Usu. +ve in Papillary RCC.
- Smooth muscle actin +ve (focal).[7]
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
- ↑ 1.0 1.1 1.2 1.3 1.4 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.
- ↑ 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.
- ↑ 3.0 3.1 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.
- ↑ 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.
- ↑ 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/.
- ↑ 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.
- ↑ 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.