Papillary renal cell carcinoma
Definition
By definition, at least 50% of the tumour shows true papillae.
Epidemiology
Papillary tumours constitute 7 to 15% of all renal cell carcinomas. There is an association with renal cortical adenomas. A hereditary form is associated with mutations of the c-met oncogene.
Radiology
Large tumours may appear cystic.
Macroscopic appearances
Small tumours may have the appearance of flecks of gold in a haemorrhagic background. There may be apparent necrosis,which is in fact due to macrophages filling the papillae.
Histopathology
The papillae may appear as spherules or tubules, lacking fibrous cores. Conversely, cores may be apparent and may be distended with macrophages.
The two most common subtypes are composed of:
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Type I: micropapillary; basophilic cells with scant cytoplasm covering short thin vascular stalks. Infiltration by foam cells is more common in this subtype3. There is a strong male predominance3.
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Type II: macropapillary; eosinophilic cells with abundant cytoplasm covering oedematous or thick stalks.
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An oncocytic variant has been proposed.
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There is also probably a solid variant8. Solid micronodules may represent abortive micropapillae8. These also show the characteristic trisomies of chromosomes 7 or 178.
Intracytoplasmic haemosiderin may be abundant. Rare psammoma bodies may be present.
Immunohistochemistry
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Pancytokeratin KL-1
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25/27(diffuse positivity of more than 80% of tumour cells; 17, strong positivity of 50-80% of tumour cells; 6, heterologous positivity of less than 50% of tumour cells; 2: study by tissue microarray)2
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34bE12
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7/27(diffuse positivity of more than 80% of tumour cells; 2, strong positivity of 50-80% of tumour cells; 4, heterologous positivity of less than 50% of tumour cells; 1: study by tissue microarray)2
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solid variant: 0/68
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Cam5.2
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25/27(diffuse positivity of more than 80% of tumour cells; 19, strong positivity of 50-80% of tumour cells; 5, heterologous positivity of less than 50% of tumour cells; 1: study by tissue microarray)2
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CK7
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12/27(diffuse positivity of more than 80% of tumour cells; 8, strong positivity of 50-80% of tumour cells; 3, heterologous positivity of less than 50% of tumour cells; 1: study by tissue microarray)2
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micropapillary:
15/15(diffuse positivity)3,
48/61(moderately or strongly positive)4
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macropapillary:
4/19(focally positive)3,6/304
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solid variant: 6/68
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CK19
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micropapillary: 15/153
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macropapillary: 10/193
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EMA
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18/27(diffuse positivity of more than 80% of tumour cells; 12, strong positivity of 50-80% of tumour cells; 3, heterologous positivity of less than 50% of tumour cells; 3: study by tissue microarray)2, 30/4611
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solid variant: 6/63
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MOC31
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3/27(diffuse positivity of more than 80% of tumour cells; 2, heterologous positivity of less than 50% of tumour cells; 1: study by tissue microarray)2
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BerEP4
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18/27(diffuse positivity of more than 80% of tumour cells; 9, strong positivity of 50-80% of tumour cells; 4, heterologous positivity of less than 50% of tumour cells; 5: study by tissue microarray)2
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RCC Ma
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17/27(diffuse positivity of more than 80% of tumour cells; 16, strong positivity of 50-80% of tumour cells; 1: study by tissue microarray)2, 13/145, 29/3012
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P504S
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41/41(22 primary type 1, 13 primary type 2, 6 metastatic type 2)14
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CD10
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16/27(diffuse positivity of more than 80% of tumour cells; 6, strong positivity of 50-80% of tumour cells; 3, heterologous positivity of less than 50% of tumour cells; 7: study by tissue microarray)2, 13/145,
19/20(moderate or extensive in 7/8 type II but in only 5/12 type I)9
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E-cadherin
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12/27(diffuse positivity of more than 80% of tumour cells; 4, strong positivity of 50-80% of tumour cells; 3, heterologous positivity of less than 50% of tumour cells; 5: study by tissue microarray)2
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CD15
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11/27(diffuse positivity of more than 80% of tumour cells; 7, strong positivity of 50-80% of tumour cells; 2, heterologous positivity of less than 50% of tumour cells; 2: study by tissue microarray)2
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Vimentin
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17/27(diffuse positivity of more than 80% of tumour cells; 8, strong positivity of 50-80% of tumour cells; 8, heterologous positivity of less than 50% of tumour cells; 1: study by tissue microarray)2, 6/76, 36/4611
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Dolichos biflorus agglutinin
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micropapillary: 11/153
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macropapillary: 2/193
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b defensin-1
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7/76
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parvalbumin
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5/76, 0/177
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kidney-specific cadherin
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5/17(staining was predominantly membranous; includes positivity in 3/7 of eosinophilic type)10, 1/4611,4/3012, 0/1513
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Papillary carcinomas frequently co-express TCC Ma and BerEP4.
Cytogenetics
Trisomies of chromosomes 7 and 17 are common.
Differential diagnosis
Prognosis
Low grade tumours have an excellent prognosis, high grade tumours a poor prognosis. Metastases are usually to lymph nodes.
References
1 Renshaw, A. A. (2002). "Subclassification of renal cell neoplasms: an update for the practising pathologist." Histopathology 41(4): 283-300.
2 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
3 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.
4 Delahunt, B. and Eble, J.N. Papillary renal cell carcinoma: a clinicopathologic and immunohistochemical study of 105 tumors. Mod Pathol 1997;10:537-44.
5 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.
6 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.
7 Martignoni, G., M. Pea, et al. (2001). "Parvalbumin is constantly expressed in chromophobe renal carcinoma." Mod Pathol 14(8): 760-7.
8 Renshaw, A.A., Zhang, H., Corless, C.L., Fletcher, J.A. and Pins, M.R. Solid variants of papillary (chromophil) renal cell carcinoma: clinicopathologic and genetic features. Am J Surg Pathol 1997;21:1203-9.
9 Langner, C., M. Ratschek, et al. (2004). "CD10 is a diagnostic and prognostic marker in renal malignancies." Histopathology 45(5): 460-7.
10 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
11 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
12 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
13 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
14 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.
©SMUHT/PW Bishop