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Kidney tumor - adult malignancies

Adult renal cell carcinoma

Classification of renal cell carcinoma

Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 3 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.


● Based on cytology, tissue characteristics (Thoenes, Storkel & Rumpelt, Path Res Pract 1986;181:125) and cytogenetics (Heidelberg classification: J Pathol 1997;183:131)
● New entities continue to be described based on immunohistochemical and molecular features – some not yet recognized in WHO Classification
● Use of “granular cell variant” not recommended – may falsely combine a spectrum of tumors with varying clinical significance / treatment: clear cell, chromophobe, unclassified renal cell carcinoma, or oncocytoma (Urology 2007;69:452)
● Diagnosis of “sarcomatoid carcinoma” not recommended; classify as high-grade carcinoma of type from which it originated (or unclassified) with sarcomatoid features and percentage of sarcomatoid features (Cancer 1997;80:987)

WHO (2004) Classification

Renal Tumors and Tumor-Like Conditions of Adults

Renal Cell Carcinoma:
● Clear cell renal cell carcinoma
● Multilocular cystic renal cell carcinoma
● Papillary renal cell carcinoma
● Chromophobe renal cell carcinoma
● Carcinoma of the collecting ducts of Bellini
● Renal medullary carcinoma
● Xp11 translocation carcinoma
● Renal cell carcinoma associated with neuroblastoma
● Mucinous tubular and spindle cell carcinoma
● Renal cell carcinoma, unclassified

Familial Renal Cell Carcinoma Syndromes:
● Von Hippel-Lindau Disease (VHL)
● Hereditary Papillary Renal Carcinoma (HPRC)
● Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC)
● Birt-Hogg-Dubé Syndrome (BHD)

Papillary (Renal Cortical) Adenoma


Metanephric Tumors:
● Metanephric adenoma
● Metanephric adenofibroma
● Metanephric stromal tumor
● Metanephric adenosarcoma

Rare Tumors with Epithelial and/or Parenchymal Differentiation:
● Carcinoid Tumor
● Neuroendocrine carcinoma of the kidney (small cell carcinoma)
● Primitive neuroectodermal tumor
● Juxtaglomerular cell tumor
● Teratoma
● Nephroblastoma and other “pediatric” renal tumors
● Cystic nephroma (multilocular cyst)
● Mixed epithelial and stromal tumor
● Spiradenocylindroma

Mesenchymal Tumors:
● Angiomyolipoma
     ● Epithelioid angiomyolipoma
● Medullary fibroma
● Leiomyoma
● Lipoma
● Hemangioma
● Lymphangioma
● Other benign mesenchymal tumors
● Leiomyosarcoma
● Liposarcoma
● Intrarenal schwannoma
● Solitary fibrous tumor
● Hemangiopericytoma
● Fibrosarcoma
● Pleomorphic sarcoma (malignant fibrous histiocytoma)
● Rhabdomyosarcoma
● Angiosarcoma
● Osteosarcoma
● Synovial sarcoma
● Other malignant mesenchymal tumors

Lymphoid Tumors:
● Lymphoma
● Plasmacytoma

Metastatic Tumors

Tumor-Like Lesions:
● Xanthogranulomatous pyelonephritis
● Inflammatory myofibroblastic tumor (pseudosarcomatous myofibroblastic proliferation)
● Perirenal and sinus cysts

Additional references

Eble: Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs (WHO, 2004), PDF

Suggested new features to incorporate:
● VEGF expression (Lab Invest 2008;88:962)
● Gene expression (ScientificWorldJournal 2006;6;2505)

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Classification of renal cell carcinoma

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