Kidney tumours

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Kidney tumours - includes malignant kidney tumours (kidney cancer) and benign kidney tumours. Medical renal diseases are dealt with in the medical renal diseases article.

A kidney tumour (renal oncocytoma). (WC/Emmanuelm)

Pediatric kidney tumours are dealt with in the pediatric kidney tumours article.

Renal specimens

In excisions done for tumours, a comment should be made about kidney distant from the tumour. People with less renal mass, i.e. less kidney, are predisposed to focal segmental glomerulosclerosis (FSGS).

Anatomy

Layers (superficial to deep):

  1. Renal fascia (Gerota's fascia).
  2. Perinephric fat.
  3. Renal capsule.
  4. Renal parenchyma (cortex).

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Missed renal biopsy

Main article: Missed renal biopsy

Tabular comparison (selected tumours)

Selected common tumours of the kidney:[2][3]

Clear cell RCC Papillary RCC
type 1 		Papillary RCC
type 2 		Chromophobe RCC
classic variant 		Chromophobe RCC
eosinophilic variant 		Oncocytoma
Gross Golden yellow, solid friable friable light brown light brown mahogany/brown, +/-central scar
Architecture nests, sheets papillary, simple papillary,
pseudostratified 		nests, sheets nests, sheets nests, sheets
Nuclear atypia low-high
typically medium-high 		low-medium medium-high low-high, "raisinoid"
nuc. membrane 		low-high, "raisinoid"
nuc. membrane 		low-medium, round nuclei
Cytoplasm clear eosinophilic eosinophilic cobwebs/clear eosinophilic/cobwebs eosinophilic/
granular & abundant
Other delicate vessels,
necrosis common 		histiocytes
in fibrovascular cores, >0.5 cm 		histiocytes
in fibrovascular cores, >0.5 cm 		perinuclear clearing, thick vessels perinuclear clearing, thick vessels in loose fibrous/hyaline stroma
IHC CK7-, EMA+ AMACR+, EMA+, CK7+ AMACR+, E-cadherin+, CK7- CD117+, CK7+ (membrane) CD117+, CK7+ (membrane) Vimentin-, EMA+
Main DDx chromophobe
classic variant 		PaRCC type 2, mets PaRCC type 1, mets clear cell RCC oncocytoma chromophobe
eosinophilic variant
Key features clear cells, vascular papillae, histiocytes
simple epithelium 		papillae, histiocytes,
stratified 		perinuc. clearing,
wispy cytoplasm 		perinuc. clearing,
wispy eosinophilic
cytoplasm 		eosinophilic, granular cytoplasm
Image(s)
 
CCRCC (WC)
 
PaRCC - intermed. (WC)
,
 
PaRCC - high (WC)
 
ChRCC (WC)
 
Oncocytoma (WC)

Notes:

  • Cell shape: all have epithelioid morphology.

Tabular comparison of oncocytoma and chromophobe RCC

Histomorphologic features useful to distinguish chromophobe RCC (eosinophilic variant) and oncocytoma:[4]

Morphologic feature ChRCC (eosinophilic
variant) 		Renal oncocytoma
Nuclear morphology "raisinoid"/wrinkled appearance round with small nucleolus,
usu. little size variation
Multinucleation common - binucleation uncommon
Chromatin coarse fine
Architecture solid, crowded nests spaced nests /
archipelago-like, solid
Cytoplasm perinuclear halo, may be focal no perinuclear halo
Degenerative foci
(focal atypia & pleomorphism) 		absent present in ~20% of cases
Image
 
ChRCC (WC/Nephron)
 
Oncocytoma (WC/Nephron)

Common DDx

Spindle cell tumours

Malignant spindle cell tumours of the kidney:

Benign spindle cell tumours of the kidney:

Renal tumours with eosinophilic cytoplasm

Main article: Renal tumours with eosinophilic cytoplasm

WHO classification of renal neoplasia

  • Based on 2004 iteration - as per WMSP, slightly modified.[5] Online, the classification can found here.

Renal cell tumours

Common:

Less common:

Metanephric tumours

Nephroblastic tumours

Mesenchymal tumours

Childhood:

Adults:

Other:

Mixed mesenchymal and epithelial tumours

Others

Vancouver modification of WHO classification

In 2012/2013, several additions were made:[6]

"Emerging" entities (as per Vancouver) are:

An entity proposed after Vancouver

Renal cell carcinoma

Overview

General

  • Relatively common form of cancer.
  • Often abbreviated RCC.
  • AKA hypernephroma.[7]
  • RCC represents approx. 90% of malignancies in kidneys of adults.[8]

Origin

  • Proximal renal tubule.

Clinical

  • Classically described as a triad:[9]
    • Hematuria (most common symptom).
    • Abdominal mass.
    • Flank pain.
  • Frequently picked-up on imaging (incidentaloma) ~ 1/3 of cases.

Risk factors

Subtypes of RCC

RCC (renal cell carcinoma) comes in different subtypes:[11]

  • Clear cell carcinoma (70-80% of RCC) -- abbrev. CCRCC.
  • Papillary renal cell carcinoma (10-15% of RCC) -- abbrev. PRCC.
  • Chromophobe renal carcinoma (5% of RCC) -- abbrev. ChRCC.
  • Collecting duct (Bellini duct) carcinoma (1% of RCC).

Notes:

  • Subtype is an independent predictor of mortality - but adds very little to multivariate models with staging information.[12]
  • CCRCC tends to be worse than ChRCC and PRCC, probably due to higher incidence of mets.[13]
  • The exam answer (worst to best): clear cell RCC, papillary RCC, chromophobe RCC.

IHC - is it RCC?

  • RCC Ma (+), CD10 (+) -- specific for RCC[14]

IHC - differentiation of types

  • Clear cell RCC vs. papillary RCC:
    • CK7 (-ve CCRCC), AMACR (+ve in PRCC).[15]
  • Papillary RCC type 1 vs. papillary RCC type 2:
    • E-cadherin +ve in PRCC type 2.[16]
    • EMA (MUC1) +ve in PRCC type 1.[16]
  • ChRCC vs. oncocytoma (ONC):
    • CK7 (ChRCC +ve membrane), CK20, CD15.[15]
    • CK7 -- ChRCC 86% +ve vs. ONC 0% +ve.[17]
    • CD15 -- ChRCC 11% +ve vs. ONC 57% +ve.[18]
    • Hale's colloidal iron +ve in ChRCC, usually neg. in ONC.[19]
    • PAX2 -- ChRCC (1/11) +ve vs. ONC (20/23) +ve.[20]
    • Kidney-specific cadherin (Ksp-cadherin) -- ChRCC 97% +ve (distinctive membrane pattern) vs. ONC only 3% +ve.[21]
  • ChRCC & renal oncocytoma vs. others:
    • CD117 (ckit) +ve (100% membrane, ~75% cytoplasmic).[22]
  • Clear cell RCC vs. chromophobe RCC:
    • Hale's colloidal iron (+ve in ChRCC).[19]
    • CK7 (cell membrane +ve in ChRCC).

Notes:

  • One paper[23] describes CD10, parvalbumin, AMACR, CK7 and S100A1 as being useful.
  • Another paper I came across:[24]
  • c-kit (CD117) not useful for differentiating ONC and ChRCC.[20]
  • E-cadherin not useful for differentiating ChRCC and ONC.[25]

RCC vs. Urothelial cell carcinoma

Main article: Urothelium
  • Clinically/radiologically, it may not be possible to differentiate renal pelvis UCC and RCC if the tumour is large.
  • Pathologically, this is not very difficult.
  • On gross specimens, it is almost always obvious what one is dealing with:

Renal cell carcinoma with sarcomatoid differentiation

  • AKA sarcomatoid renal cell carcinoma.
Main article: Renal cell carcinoma with sarcomatoid differentiation

Renal cell carcinoma with rhabdoid morphology

  • AKA renal cell carcinoma with rhabdoid change.
Main article: Renal cell carcinoma with rhabdoid morphology

Hereditary renal cell carcinoma

The classics - which are all autosomal dominant:[11]

  1. Von Hippel-Lindau syndrome.
    • VHL gene mutation.
    • Clear cell RCC.
  2. Hereditary clear cell renal cell carcinoma.
    • VHL gene mutation.
  3. Hereditary papillary renal cell carcinoma.
    • MET proto-oncogene mutation.
    • PaRCC type 1.[26]
  4. Hereditary leiomyomatosis and renal cell cancer:[26]
  5. Birt–Hogg–Dubé syndrome:[26]
    • FLCN (folliculin) gene mutation.[28]
    • Skin lesions (fibrofolliculoma, trichodiscoma, acrochordon).
    • ChRCC most common, other types seen (e.g. oncocytoma).
    • Variable penetrance (autosomal dominant).

Others:

  • Hereditary papillary carcinoma (TFE3 related translocations).[29]

Notes:

  • A total of ten hereditary renal cancer syndromes have been described. In eight of the ten the gene is known.[30]

Molecular

Recurrent molecular changes in RCC:

  • Clear cell RCC:
    • Loss of 3p - contains the VHL gene.
  • Papillary RCC:
    • Sporadic:
      • Trisomy 7, 16, 17.
      • Loss of Y.
    • Familial:
      • Trisomy 7 - contains MET gene.[31]

Renal cell carcinoma grading

Main article: Renal cell carcinoma grading

Renal cell carcinoma staging

Main article: Kidney cancer staging

Renal sinus invasion

Main article: Kidney cancer staging

Clear cell renal cell carcinoma

Main article: Clear cell renal cell carcinoma

Multilocular cystic renal cell carcinoma

Main article: Multilocular cystic renal cell carcinoma

Papillary renal cell carcinoma

Main article: Papillary renal cell carcinoma

Chromophobe renal cell carcinoma

Main article: Chromophobe renal cell carcinoma

Clear cell papillary renal cell carcinoma

Main article: Clear cell papillary renal cell carcinoma

Unclassified renal cell carcinoma

  • Abbreviated URCC.
Main article: Unclassified renal cell carcinoma

Renal translocation carcinomas

Renal tumour with Xp11.2 translocation

Main article: Renal tumour with Xp11.2 translocation

Renal tumour with t(6;11) translocation

  • AKA t(6;11) renal cell carcinoma.
Main article: Renal tumour with t(6;11) translocation

Benign tumours

Papillary adenoma of the kidney

  • AKA renal papillary adenoma.
Main article: Papillary adenoma of the kidney

Renal oncocytoma

Main article: Renal oncocytoma

Angiomyolipoma

  • Abbreviated AML.
Main article: Angiomyolipoma

Mimics

Xanthogranulomatous pyelonephritis

  • Abbreviated XGP.
Main article: Xanthogranulomatous pyelonephritis

Malakoplakia

Main article: Malakoplakia

Rare stuffs

Juxtaglomerular cell tumour

  • AKA juxtaglomerular tumour, reninoma.[32]
Main article: Juxtaglomerular cell tumour

Renomedullary interstitial cell tumour

Main article: Renomedullary interstitial cell tumour

Metanephric adenoma

  • Should not be confused mesonephric adenoma, another term for nephrogenic adenoma.
    • Memory device: metanephric adenoma is a tumour.
Main article: Metanephric adenoma

Renal epithelial and stromal tumour

Abbreviated REST.

The lumping term for both:[34]

  1. Mixed epithelial and stromal tumour.
  2. Cystic nephroma.

Mixed epithelial and stromal tumour

  • Abbreviated MEST.
Main article: Mixed epithelial and stromal tumour of the kidney

Cystic nephroma

Main article: Cystic nephroma

Renal mucinous tubular and spindle cell carcinoma

  • AKA renal mucinous tubular spindle cell carcinoma.
  • AKA mucinous tubular and spindle cell carcinoma of the kidney.[35]
Main article: Renal mucinous tubular and spindle cell carcinoma

Collecting duct carcinoma

Main article: Collecting duct carcinoma

Renal medullary carcinoma

Main article: Renal medullary carcinoma

Tubulocystic carcinoma of the kidney

Main article: Tubulocystic carcinoma of the kidney

Acquired cystic disease-associated renal cell carcinoma

Main article: Acquired cystic disease-associated renal cell carcinoma

Kidney metastasis

  • AKA renal metastasis, metastatic kidney disease.
Main article: Kidney metastasis

Pediatric

Main article: Pediatric kidney tumours

The most common is nephroblastoma (Wilms tumour).

Others include:

See also

References

  1. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 288. ISBN 978-0781765275.
  2. ↑ Zhou, Ming; Magi-Galluzzi, Cristina (2006). Genitourinary Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 281-304. ISBN 978-0443066771.
  3. ↑ 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.
  4. ↑ Tickoo, SK.; Amin, MB. (Dec 1998). "Discriminant nuclear features of renal oncocytoma and chromophobe renal cell carcinoma. Analysis of their potential utility in the differential diagnosis.". Am J Clin Pathol 110 (6): 782-7. PMID 9844591.
  5. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 291. ISBN 978-0781765275.
  6. ↑ 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.
  7. ↑ URL:http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001544/. Accessed on: 14 July 2011.
  8. ↑ 8.0 8.1 8.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 289. ISBN 978-0781765275.
  9. ↑ Schmid HP, Szabo J (May 1997). "[Renal cell carcinoma--a current review]" (in German). Praxis (Bern 1994) 86 (20): 837?3. PMID 9312811.
  10. ↑ Chow, WH.; McLaughlin, JK.; Mandel, JS.; Wacholder, S.; Niwa, S.; Fraumeni, JF. (Jan 1996). "Obesity and risk of renal cell cancer.". Cancer Epidemiol Biomarkers Prev 5 (1): 17-21. PMID 8770461.
  11. ↑ 11.0 11.1 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.
  12. ↑ Capitanio, U.; Cloutier, V.; Zini, L.; Isbarn, H.; Jeldres, C.; Shariat, SF.; Perrotte, P.; Antebi, E. et al. (Jun 2009). "A critical assessment of the prognostic value of clear cell, papillary and chromophobe histological subtypes in renal cell carcinoma: a population-based study.". BJU Int 103 (11): 1496-500. doi:10.1111/j.1464-410X.2008.08259.x. PMID 19076149.
  13. ↑ Delahunt, B.; Bethwaite, PB.; Nacey, JN. (Oct 2007). "Outcome prediction for renal cell carcinoma: evaluation of prognostic factors for tumours divided according to histological subtype.". Pathology 39 (5): 459-65. doi:10.1080/00313020701570061. PMID 17886093.
  14. ↑ Zhou M, Roma A, Magi-Galluzzi C (June 2005). "The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms". Clin. Lab. Med. 25 (2): 247?7. doi:10.1016/j.cll.2005.01.004. PMID 15848735.
  15. ↑ 15.0 15.1 Zhou M, Roma A, Magi-Galluzzi C (June 2005). "The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms". Clin. Lab. Med. 25 (2): 247?7. doi:10.1016/j.cll.2005.01.004. PMID 15848735.
  16. ↑ 16.0 16.1 Langner C, Ratschek M, Rehak P, Schips L, Zigeuner R (February 2004). "Expression of MUC1 (EMA) and E-cadherin in renal cell carcinoma: a systematic immunohistochemical analysis of 188 cases". Mod. Pathol. 17 (2): 180?. doi:10.1038/modpathol.3800032. PMID 14657952.
  17. ↑ Liu L, Qian J, Singh H, Meiers I, Zhou X, Bostwick DG (August 2007). "Immunohistochemical analysis of chromophobe renal cell carcinoma, renal oncocytoma, and clear cell carcinoma: an optimal and practical panel for differential diagnosis". Arch. Pathol. Lab. Med. 131 (8): 1290?. PMID 17683191. http://journals.allenpress.com/jrnlserv/?request=get-abstract&issn=0003-9985&volume=131&page=1290.
  18. ↑ Pan CC, Chen PC, Ho DM (November 2004). "The diagnostic utility of MOC31, BerEP4, RCC marker and CD10 in the classification of renal cell carcinoma and renal oncocytoma: an immunohistochemical analysis of 328 cases". Histopathology 45 (5): 452?. doi:10.1111/j.1365-2559.2004.01962.x. PMID 15500648.
  19. ↑ 19.0 19.1 Geramizadeh B, Ravanshad M, Rahsaz M (2008). "Useful markers for differential diagnosis of oncocytoma, chromophobe renal cell carcinoma and conventional renal cell carcinoma". Indian J Pathol Microbiol 51 (2): 167?1. PMID 18603673. http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2008;volume=51;issue=2;spage=167;epage=171;aulast=Geramizadeh.
  20. ↑ 20.0 20.1 Memeo L, Jhang J, Assaad AM, et al. (February 2007). "Immunohistochemical analysis for cytokeratin 7, KIT, and PAX2: value in the differential diagnosis of chromophobe cell carcinoma". Am. J. Clin. Pathol. 127 (2): 225–9. doi:10.1309/9KWEA4W9Y94D1AEE. PMID 17210525. http://ajcp.ascpjournals.org/cgi/pmidlookup?view=long&pmid=17210525.
  21. ↑ Mazal PR, Exner M, Haitel A, et al. (January 2005). "Expression of kidney-specific cadherin distinguishes chromophobe renal cell carcinoma from renal oncocytoma". Hum. Pathol. 36 (1): 22–8. doi:10.1016/j.humpath.2004.09.011. PMID 15712178.
  22. ↑ Krueger S, Sotlar K, Kausch I, Horny HP (2005). "Expression of KIT (CD117) in renal cell carcinoma and renal oncocytoma". Oncology 68 (2-3): 269-75. doi:10.1159/000086783. PMID 16015044.
  23. ↑ Martignoni G, Brunelli M, Gobbo S, et al (February 2007). "Role of molecular markers in diagnosis and prognosis of renal cell carcinoma". Anal. Quant. Cytol. Histol. 29 (1): 41?. PMID 17375873.
  24. ↑ Avery AK, Beckstead J, Renshaw AA, Corless CL (February 2000). "Use of antibodies to RCC and CD10 in the differential diagnosis of renal neoplasms". Am. J. Surg. Pathol. 24 (2): 203?0. PMID 10680888. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0147-5185&volume=24&issue=2&spage=203.
  25. ↑ Kim MK, Kim S (December 2002). "Immunohistochemical profile of common epithelial neoplasms arising in the kidney". Appl. Immunohistochem. Mol. Morphol. 10 (4): 332–8. PMID 12613443.
  26. ↑ 26.0 26.1 26.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 290. ISBN 978-0781765275.
  27. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 136850
  28. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 135150
  29. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 314310
  30. ↑ Verine, J.; Pluvinage, A.; Bousquet, G.; Lehmann-Che, J.; de Bazelaire, C.; Soufir, N.; Mongiat-Artus, P. (Nov 2010). "Hereditary renal cancer syndromes: an update of a systematic review.". Eur Urol 58 (5): 701-10. doi:10.1016/j.eururo.2010.08.031. PMID 20817385.
  31. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 164860
  32. ↑ Wong, L.; Hsu, TH.; Perlroth, MG.; Hofmann, LV.; Haynes, CM.; Katznelson, L. (Feb 2008). "Reninoma: case report and literature review.". J Hypertens 26 (2): 368-73. doi:10.1097/HJH.0b013e3282f283f3. PMID 18192852.
  33. ↑ Bircan, S.; Orhan, D.; Tulunay, O.; Safak, M. (2000). "Renomedullary interstitial cell tumor.". Urol Int 65 (3): 163-6. PMID 11054036.
  34. ↑ Turbiner, J.; Amin, MB.; Humphrey, PA.; Srigley, JR.; De Leval, L.; Radhakrishnan, A.; Oliva, E. (Apr 2007). "Cystic nephroma and mixed epithelial and stromal tumor of kidney: a detailed clinicopathologic analysis of 34 cases and proposal for renal epithelial and stromal tumor (REST) as a unifying term.". Am J Surg Pathol 31 (4): 489-500. doi:10.1097/PAS.0b013e31802bdd56. PMID 17414095.
  35. ↑ Brandal, P.; Lie, AK.; Bassarova, A.; Svindland, A.; Risberg, B.; Danielsen, H.; Heim, S. (Feb 2006). "Genomic aberrations in mucinous tubular and spindle cell renal cell carcinomas.". Mod Pathol 19 (2): 186-94. doi:10.1038/modpathol.3800499. PMID 16258504.

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