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 classification.

2003 WHO Classification of Tumours of the Urinary System and Male Genital Organs
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, 2001.

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

Type 1
Type 2
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

Classic
Eosinophilic variant
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. 118:877-886, 2002.

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, 1999.

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.

Papillary adenoma
Grignon and Eble: Papillary and metanephric adenomas of the kidney. Semin.Diagn.Pathol. 15:41-53, 1998.

Oncocytoma
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.

Metanephric Neoplasia

Metanephric adenoma
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

1. Guinan, Vogelzang, et al.: Renal cell carcinoma: tumor size, stage and survival. J.Urol. 153:901-903, 1995.
2. 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.
3. 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. 162:1930-1933, 1999.
4. 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. 163:1090-1095, 2000.
5. T3 Tumor extends into major veins or invades adrenal gland or perinephric tissues but not beyond Gerota's fascia
6. T3a Tumor directly invades adrenal gland or perirenal and/or renal sinus fat but not beyond Gerota's fascia
7. T3b Tumor grossly extends into renal vein or its segmental (muscle-containing) branches or vena cava below diaphragm
8. T3c Tumor grossly extends into vena cava above diaphragm or invades the wall of the vena cava
9. T4 Tumor invades beyond Gerota's fascia