The numerous recent advances in our understanding of renal cell neoplasia are reflected
in the 2002 AJCC staging system which replaces the 1997 edition, and in the 2003 WHO classification of
tumors of the urinary and male genital system which replaces the 1998 classification.
This presentation is intended to highlight the most important developments and this
handout directs the reader to the publications which, in my opinion, have been most influential in
prompting the changes and are most helpful in facilitating the diagnosis of the entities in the new
2003 WHO Classification of Tumours of the Urinary System and Male Genital
Epithelial Neoplasms of the Renal Cortex and Medulla
Renal Cell Neoplasia
Clear cell renal cell carcinoma
Delahunt, Kittelson, et al.: Prognostic importance of tumor size for localized conventional (clear
cell) renal cell carcinoma, assessment of TNM T1 and T2 tumor categories and comparison with other
prognostic parameters. Cancer 94:658-664, 2002.
Cheville, Blute, et al.: Stage pT1 conventional (clear cell) renal cell carcinoma: pathological
features associated with cancer specific survival. J.Urol. 166:453-456,
Multilocular clear cell renal cell carcinoma
Eble and Bonsib. Extensively cystic renal neoplasms: cystic nephroma, cystic partially
differentiated nephroblastoma, multilocular cystic renal cell carcinoma, and cystic hamartoma of renal
pelvis. Semin.Diagn.Pathol. 15:2-20, 1998.
Papillary renal cell carcinoma
Delahunt, Eble, et al.: Morphologic typing of papillary renal cell carcinoma: comparison of growth
kinetics and patient survival in 66 cases. Hum.Pathol. 32:590-595, 2001.
Leroy, Zini, et al. Morphologic subtyping of papillary renal cell carcinoma: correlation with
prognosis and differential expression of MUC1 between the two subtypes.
Mod.Pathol. 15:1126-1130, 2002.
Jiang, Richter, et al.: Chromosomal imbalances in papillary renal cell carcinoma: genetic
differences between histologic subtypes. Am.J.Pathol. 153:1467-1473, 1998.
Chromophobe renal cell carcinoma
Amin, Amin, et al.: Prognostic impact of histologic subtyping of adult renal epithelial neoplasms,
an experience of 405 cases. Am.J.Surg.Pathol. 26:281-291, 2002.
Lohse, Blute, et al.: Comparison of standardized and nonstandardized nuclear grade of renal cell
carcinoma to predict outcome among 2,042 patients. Am.J.Clin.Pathol.
Carcinoma of the collecting ducts of Bellini
Srigley and J. N. Eble. Collecting duct carcinoma of kidney. Semin.Diagn.Pathol. 15:54-67, 1998.
Renal medullary carcinoma
Davis, Mostofi, et al.: Renal medullary carcinoma: the seventh sickle cell nephropathy. Am.J.Surg.Pathol. 19:1-11, 1995.
Xp11 translocation carcinomas
Argani, Antonescu, et al.: PRCC-TFE3 renal carcinomas: morphologic,
immunohistochemical, ultrastructural, and molecular analysis of an entity associated with the
t(X;1)(p11.2;q21). Am.J.Surg.Pathol. 26:1553-1566, 2002.
Argani, Antonescu, et al.: Primary renal neoplasms with the ASPL-TFE3
gene fusion of alveolar soft part sarcoma: a distinctive tumor entity previously included among renal
cell carcinomas of children and adolescents. Am.J.Pathol. 159:179-192, 2001.
Heimann, El Housni, et al.: Fusion of a novel gene, RCC17, to the TFE3 gene in t(X;17) (p11.2;q25.3)-bearing papillary renal cell carcinomas. Cancer Res. 61:4130-4135, 2001.
Carcinoma associated with neuroblastoma
Medeiros, Palmedo, et al.: Oncocytoid renal cell carcinoma after neuroblastoma: a report of four
cases of a distinct clinicopathologic entity. Am.J.Surg.Pathol. 23:772-780,
Mucinous tubular and spindle cell carcinoma
Parwani, Husain, et al.: Low-grade myxoid renal epithelial neoplasms with distal nephron
differentiation. Hum.Pathol. 32:506-512, 2001.
Rakozy, Schmahl, et al.: Low-grade tubular-mucinous renal neoplasms: morphologic,
immunohistochemical, and genetic features. Mod.Pathol. 15:1162-1171, 2002.
Renal cell carcinoma, unclassified
Störkel, Eble, et al.: Classification of renal cell carcinoma, workgroup 1.
Cancer 80:987-989, 1997.
Grignon and Eble: Papillary and metanephric adenomas of the kidney.
Semin.Diagn.Pathol. 15:41-53, 1998.
Perez-Ordonez, Hamed, et al. Renal oncocytoma: a clinicopathologic study of 70 cases. Am.J.Surg.Pathol. 21:871-883, 1997.
Amin, Crotty, et al. Renal oncocytoma: a reappraisal of morphologic features with clinicopathologic
findings in 80 cases. Am.J.Surg.Pathol. 21:1-12, 1997.
Muir, Cheville, et al.: Metanephric adenoma, nephrogenic rests, and Wilms' tumor: a histologic and
immunophenotypic comparison. Am.J.Surg.Pathol. 25:1290-1296, 2001.
2002 AJCC Staging System for Renal Tumors
- T1 Tumor 7cm or less in greatest dimension and confined to the kidney
- T1a Tumor 4cm or less in greatest dimension and confined to the kidney
- T1b Tumor more than 4 cm but not more than 7 cm and confined to the kidney
- T2 Tumor more than 7cm in greatest dimension and confined to the kidney
- Guinan, Vogelzang, et al.: Renal cell carcinoma: tumor size, stage and survival. J.Urol. 153:901-903, 1995.
- Lerner, Hawkins, et al.: Disease outcome in patients with low stage renal cell carcinoma treated
with nephron sparing or radical surgery. J.Urol. 155:1868-1873, 1996.
- Hafez, Fergany, et al.: Nephron sparing surgery for localized renal cell carcinoma: impact of
tumor size on patient survival, tumor recurrence and TNM staging. J.Urol.
- Tsui, Shvarts, et al.: Prognostic indicators for renal cell carcinoma: a multivariate analysis of
643 patients using the revised 1997 TNM staging criteria. J.Urol.
- T3 Tumor extends into major veins or invades adrenal gland or perinephric tissues but not beyond
- T3a Tumor directly invades adrenal gland or perirenal and/or renal sinus fat but not beyond Gerota's
- T3b Tumor grossly extends into renal vein or its segmental (muscle-containing) branches or vena cava
- T3c Tumor grossly extends into vena cava above diaphragm or invades the wall of the vena cava
- T4 Tumor invades beyond Gerota's fascia