Papillary renal cell carcinoma
Papillary renal cell carcinoma | |
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Diagnosis in short | |
![]() Papillary renal cell carcinoma. H&E stain. | |
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LM | cuboidal or low columnar cells (simple or pseudostratified) on papillae, interstitial foam cells in the vascular cores |
Subtypes | type 1, type 2, oncocytic variant |
LM DDx | clear cell renal cell carcinoma, clear cell papillary renal cell carcinoma, metanephric adenoma (esp. solid PRCC type 1), collecting duct carcinoma (esp. PRCC type 2), renal papillary adenoma, acquired cystic disease-associated renal cell carcinoma, urothelial carcinoma, renal mucinous tubular and spindle cell carcinoma |
Gross | may be multifocal, must be >0.5 cm (otherwise renal papillary adenoma) |
Site | kidney - see kidney tumours |
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Associated Dx | acquired renal cystic disease (end-stage renal disease) |
Syndromes | hereditary papillary renal cell carcinoma |
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Prevalence | relatively common |
Clin. DDx | other kidney tumours |
Treatment | surgical excision, ablation |
Papillary renal cell carcinoma, abbreviated PRCC, PaRCC and papillary RCC, is the second most common type of renal cell carcinoma.
General
- Often subclassified[1] into type 1 and type 2 -- see microscopic.
- Type 1 and Type 2 are different on a cytogenetic and molecular basis.[2]
Epidemiology
- Associated with acquired renal cystic disease.[3]
- May be familial - uncommon.[4]
- MET mutation[5] - autosomal dominant transmission, PaRCC type 1.
Gross
- Renal cortical mass > 0.5 cm.
- May be multifocal.
Microscopic
Features:[6]
- Cuboidal or low columnar cell in papillae.
- Interstitial foam cells in vascular cores - key feature.
- Most sensitive and specific feature of PRCC.[7]
- Highly vascular.
Size criterion:
- Papillary lesions must be >0.5 cm to be called carcinoma; smaller lesions (<=0.5 cm) are called papillary adenomas.[8]
Mnemonic HIP: highly vascular, interstitial foam cells, papillae.
DDx:
- Clear cell RCC.
- Papillary: histiocytes, intracellular hemosiderin, CK7 +ve.
- Clear cell papillary renal cell carcinoma - apical nuclei, usu. no true papillae.
- Metanephric adenoma (esp. solid PRCC type 1) - no histiocytes, WT-1 +ve.
- Collecting duct carcinoma - esp. PRCC type 2.
- Urothelial carcinoma.
- Renal papillary adenoma - doesn't fulfill size criterion for PaRCC.
- Acquired cystic disease-associated renal cell carcinoma.
- Renal mucinous tubular and spindle cell carcinoma.
Images
Histological subtyping
Generally accepted subtypes:[1][9]
- Type 1 - single layer of cells on basement membrane - most important.
- Usually low grade nuclear features, i.e. low Fuhrman grade.
- Other characteristics:
- Clear cytoplasm.
- Foamy macrophages - common.
- Cells smaller.
- Type 2 - pseudostratification of cells - most important.
- Usually high grade nuclear features, i.e. high Fuhrman grade.
- Other characteristics:
- Eosinophilic cytoplasm.
- Foamy macrophages - uncommon.
- Cells larger.
Another subtype:
- Oncocytic - oncocytic cytoplasm.
- Extremely rare ~ largest series is 12 cases.[10]
IHC
Features:[1]
- AMACR +ve.
- HMWCK (34betaE12) +ve.
- Panker (AE1/AE3) +ve.
- CK7 +ve ~90% of type 1, 20% of type 2.
More reading:
Type 1 versus Type 2:[11]
- CK7:
- Type 1 ~ 100%.
- Type 2 ~ 19%.
- CK19:
- Type 1 ~ 100%.
- Type 2 ~ 53%.
Metanephric adenoma vs. PaRCC type 1:[12]
- AMACR +ve.
- WT-1 -ve.
- CD57 -ve.
Molecular
Features:[13]
- Sporadic: trisomies 7, 16, 17.
- Familial: trisomy 7.
- Chromosome 7 = location of MET gene.
Note:
- Not used for diagnosis.[14]
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KIDNEY, RIGHT, RADICAL NEPHRECTOMY: - PAPILLARY RENAL CELL CARCINOMA, TYPE 1, FUHRMANN GRADE 3, pT2a(2), pNx. -- SURGICAL MARGINS NEGATIVE. -- PLEASE SEE TUMOUR SUMMARY. - RENAL PAPILLARY ADENOMAS.
Micro
The sections show a tumour in the kidney with fibrovascular cores (papillae) that focally contain macrophages. Psammoma bodies are present. Siderophages are present.
The papillae predominantly have a single layer of tumour cells and the cytoplasm of the tumour cells is predominantly clear.
Nucleoli are visible focally with the 10x objective (Fuhrman grade 3).
A second tumour with the same morphology is present and measures 8 millimetres.
Multiple small lesions, like the largest tumour, less than 0.5 cm are present.
Oncocytic variant
KIDNEY, RIGHT, NEPHRECTOMY: - PAPILLARY RENAL CELL CARCINOMA, ONCOCYTIC -- SEE COMMENT; - FUHRMANN GRADE 2; - SURGICAL MARGINS NEGATIVE; - PLEASE SEE TUMOUR SUMMARY. COMMENT: The oncocytic variant of papillary renal cell carcinoma (RCC) is uncommon and not widely recognized as a subtype of papillary RCC. The prognostic significance of the oncocytic cytoplasm is uncertain.[1] The histomorphology in this case is compatible with a type 1 papillary RCC. 1. Ann Diagn Pathol. 2006 Jun;10(3):133-9.
See also
References
- ↑ 1.0 1.1 1.2 Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 289. ISBN 978-0443066771.
- ↑ Klatte, T.; Pantuck, AJ.; Said, JW.; Seligson, DB.; Rao, NP.; LaRochelle, JC.; Shuch, B.; Zisman, A. et al. (Feb 2009). "Cytogenetic and molecular tumor profiling for type 1 and type 2 papillary renal cell carcinoma.". Clin Cancer Res 15 (4): 1162-9. doi:10.1158/1078-0432.CCR-08-1229. PMID 19228721.
- ↑ Fogo, Agnes B.; Kashgarian, Michael (2005). Diagnostic Atlas of Renal Pathology: A Companion to Brenner and Rector's The Kidney 7E (1st ed.). Saunders. pp. 438. ISBN 978-1416028710.
- ↑ Czene, K.; Hemminki, K. (Apr 2003). "Familial papillary renal cell tumors and subsequent cancers: a nationwide epidemiological study from Sweden.". J Urol 169 (4): 1271-5. doi:10.1097/01.ju.0000052373.36963.12. PMID 12629341.
- ↑ Wadt, KA.; Gerdes, AM.; Hansen, TV.; Toft, BG.; Friis-Hansen, L.; Andersen, MK. (Sep 2012). "Novel germline c-MET mutation in a family with hereditary papillary renal carcinoma.". Fam Cancer 11 (3): 535-7. doi:10.1007/s10689-012-9542-6. PMID 22717761.
- ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1017-8. ISBN 0-7216-0187-1.
- ↑ Granter SR, Perez-Atayde AR, Renshaw AA (October 1998). <303::AID-CNCR6>3.0.CO;2-7 "Cytologic analysis of papillary renal cell carcinoma". Cancer 84 (5): 303?8. PMID 9801205. http://dx.doi.org/10.1002/(SICI)1097-0142(19981025)84:5<303::AID-CNCR6>3.0.CO;2-7.
- ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 288. ISBN 978-0443066771.
- ↑ Delahunt, B.; Eble, JN. (Jun 1997). "Papillary renal cell carcinoma: a clinicopathologic and immunohistochemical study of 105 tumors.". Mod Pathol 10 (6): 537-44. PMID 9195569.
- ↑ Srigley, JR.; Delahunt, B. (Jun 2009). "Uncommon and recently described renal carcinomas.". Mod Pathol 22 Suppl 2: S2-S23. doi:10.1038/modpathol.2009.70. PMID 19494850.
- ↑ Ono, Y.; Ito, T.; Tsujino, S.; Aizawa, S.; Suzuki, M. (Jun 1997). "[A study of papillary renal cell carcinoma. Clinicopathological, immunohistochemical features and its typing].". Nihon Hinyokika Gakkai Zasshi 88 (6): 587-95. PMID 9234615.
- ↑ Watanabe, S.; Naganuma, H.; Shimizu, M.; Ota, S.; Murata, S.; Nihei, N.; Matsushima, J.; Mikami, S. et al. (2013). "Adult nephroblastoma with predominant epithelial component: a differential diagnostic candidate of papillary renal cell carcinoma and metanephric adenoma-report of three cases.". Case Rep Pathol 2013: 675875. doi:10.1155/2013/675875. PMID 24083046.
- ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1016. ISBN 0-7216-0187-1.
- ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 292. ISBN 978-0781765275.