Renal clear cell carcinoma, conventional renal cell carcinoma

Epidemiology

Renal clear cell carcinomas form 75% of all renal cell carcinomas.

This type of tumour arises in patient with the von Hippel-Lindau syndrome.

Macroscopic appearances

Histopathology

Cytoplasm may be clear or granular. There may be vacuolation, with the vacuoles sited peripherally. There is copious cytoplasm with a central nucleus with a prominent nucleolus. Sometimes the nuclei are eccentric, giving a plasmacytoid appearance.

Immunohistochemistry

 

Pancytokeratin KL-1

218/2564

 

34bE12

4/2564

Cam5.2

223/2564

CK7

16/2564, 1/185

EMA

197/2564, 55/10216

MOC31

11/2564

BerEP4

68/2564

RCC Ma

123/2564, 53/626, 5/613, 36/4217

CD10

209/2564, 58/626, 124/12912, 6/613

 

E-cadherin

132/2564

CD15

153/2504

Vimentin

164/2564, 19/238, 67/10216

CD117

4/817, 0/613

b defensin-1

3/238

parvalbumin

5/238, 0/759

RON

3/613

anti-mitochondrial antibody

diffuse in eosinophilic variant11

Colloidal iron

0/613

kidney-specific cadherin

6/3615, 0/10216, 6/4217, 10/3318

P504S

13/5219

   

Cytogenetics

Deletions of chromosome 3p are common, the site of the von Hippel-Lindau gene.

Differential diagnosis of primary renal tumours:

Differential diagnosis of metastatic clear cell renal cell carcinoma2:

Usually coexpress pancytokeratin and vimentin, unlike most other carcinomas. Most are EMA positive but negative for CEA and for both CK7 and CK203. However, there is considerable overlap with other tumours which constitute differentials of renal cell carcinoma. The "Renal cell carcinoma marker" has a promising specificity for renal cell carcinoma.

metastatic renal carcinoma:

 

coexpression of cytokeratin and vimentin

 

versus

most other carcinomas:

cytokeratin positive, vimentin negative

 

metastasis to adrenal:

 

EMA positive, cytokeratin positive

 

versus

primary adrenal cortical carcinoma:

EMA negative, only weakly cytokeratin positive.

 

metastasis to thyroid

 

thyroglobulin negative

 

versus

primary clear cell carcinoma of thyroid:

thyroglobulin positive

 

metastasis to brain:

 

EMA positive

versus

primary capillary haemangioblastoma or CNS:

EMA negative

 

metastasis to pleura

versus

renal cell carcinoma

 

Angioleiomyoma-like proliferations have been reported adjacent to renal clear cell carcinoma14, with a differential diagnosis that includes:

 

Prognosis

There is a propensity to metastasis to unusual sites. The prognosis of the cystic variant is excellent1.

References

1 Renshaw, A. A. (2002). "Subclassification of renal cell neoplasms: an update for the practising pathologist." Histopathology 41(4): 283-300.

2 Diagnostic histopathology of tumors. Edited by CDM Fletcher. 2nd edition. Churchill Livingstone. Page 485.

3 McGregor, D. K., Khurana, K. K., Cao, C. Diagnosing primary and metastatic renal cell carcinoma: the use of the monoclonal antibody 'Renal Cell Carcinoma Marker'. Am J Surg Pathol 2001;25:1485-1492.

4 Pan CC, Chen PC,Ho DM. 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 2004; 45:452-9

5 Mathers ME, Pollock AM, Marsh C, et al. Cytokeratin 7: a useful adjunct in the diagnosis of chromophobe renal cell carcinoma. Histopathology 2002; 40:563-7

6 Avery, A.K., Beckstead, J., Renshaw, A.A. and Corless, C.L. Use of antibodies to RCC and CD10 in the differential diagnosis of renal neoplasms. Am J Surg Pathol 2000;24:203-10.

7 Ono, Y., Ito, T., Tsujino, S., Aizawa, S. and Suzuki, M. [A study of papillary renal cell carcinoma. Clinicopathological, immunohistochemical features and its typing]. Nippon Hinyokika Gakkai Zasshi 1997;88:587-95.

8 Young, A.N., de Oliveira Salles, P.G., Lim, S.D., Cohen, C., Petros, J.A., Marshall, F.F., Neish, A.S. and Amin, M.B. Beta defensin-1, parvalbumin, and vimentin: a panel of diagnostic immunohistochemical markers for renal tumors derived from gene expression profiling studies using cDNA microarrays. Am J Surg Pathol 2003;27:199-205.

9 Martignoni, G., M. Pea, et al. (2001). "Parvalbumin is constantly expressed in chromophobe renal carcinoma." Mod Pathol 14(8): 760-7.

10 11 Abrahams, N. A., G. T. Maclennan, et al. (2004). "Chromophobe renal cell carcinoma: a comparative study of histological, immunohistochemical and ultrastructural features using high throughput tissue microarray." Histopathology 45(6): 593-602.

12 Langner, C., M. Ratschek, et al. (2004). "CD10 is a diagnostic and prognostic marker in renal malignancies." Histopathology 45(5): 460-7.

13 Wang HY,Mills SE. KIT and RCC are useful in distinguishing chromophobe renal cell carcinoma from the granular variant of clear cell renal cell carcinoma. Am J Surg Pathol 2005; 29:640-6

14 Kuhn E, De Anda J, Manoni S, et al. Renal cell carcinoma associated with prominent angioleiomyoma-like proliferation: Report of 5 cases and review of the literature. Am J Surg Pathol 2006; 30:1372-81

15 Kuehn A, Paner GP, Skinnider BF, et al. Expression analysis of kidney-specific cadherin in a wide spectrum of traditional and newly recognized renal epithelial neoplasms: diagnostic and histogenetic implications. Am J Surg Pathol 2007; 31:1528-33

16 Mazal PR, Exner M, Haitel A, et al. Expression of kidney-specific cadherin distinguishes chromophobe renal cell carcinoma from renal oncocytoma. Hum Pathol 2005; 36:22-8

17 Shen SS, Krishna B, Chirala R, et al. Kidney-specific cadherin, a specific marker for the distal portion of the nephron and related renal neoplasms. Mod Pathol 2005; 18:933-40 FULL TEXT

18 Adley BP, Gupta A, Lin F, et al. Expression of kidney-specific cadherin in chromophobe renal cell carcinoma and renal oncocytoma. Am J Clin Pathol 2006; 126:79-85

19 Tretiakova MS, Sahoo S, Takahashi M, et al. Expression of alpha-methylacyl-CoA racemase in papillary renal cell carcinoma. Am J Surg Pathol 2004; 28:69-76

 

This page last revised 2.2.2008.

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