Kidney tumor
Adult renal cell carcinoma
Classification of renal cell carcinoma

Topic Completed: 1 December 2016

Revised: 13 December 2019

Copyright: 2003-2019,, Inc.

PubMed search: "WHO classification" renal cell carcinoma adult

Nicole K. Andeen, M.D.
Maria Tretiakova, M.D., Ph.D.
Page views in 2019: 8,571
Page views in 2020 to date: 1,297
Cite this page: Andeen NK, Tretiakova M. Classification of renal cell carcinoma. website. Accessed February 27th, 2020.
Definition / general
  • Based on cytologic, architectural, immunohistochemical, cytogenetic and molecular characteristics.
  • 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 (2016) Classification
Tumors of the kidney
Renal Cell Tumors
  • Clear cell renal cell carcinoma
  • Multilocular cystic renal neoplasm of low malignant potential
  • Papillary renal cell carcinoma
  • Hereditary leiomyomatosis and renal cell carcinoma associated renal cell carcinoma
  • Chromophobe renal cell carcinoma
  • Collecting duct carcinoma
  • Renal medullary carcinoma
  • MiT family translocation renal cell carcinomas
  • Succinate dehydrogenase deficient renal cell carcinoma
  • Mucinous tubular and spindle cell carcinoma
  • Tubulocystic renal cell carcinoma
  • Acquired cystic disease-associated renal cell carcinoma
  • Clear cell papillary renal cell carcinoma
  • Renal cell carcinoma, unclassified
  • Papillary adenoma
  • Oncocytoma

Metanephric tumors
  • Metanephric adenoma
  • Metanephric adenofibroma
  • Metanephric stromal tumor

Nephroblastic and cystic tumors occurring mainly in children - refer to Pediatric classification

Mesenchymal tumors
  • Mesenchymal tumors occurring mainly in children – refer to Pediatric classification
  • Mesenchymal tumors occurring mainly in adults
    • Leiomyosarcoma (including renal vein leiomyosarcoma)
    • Angiosarcoma
    • Rhabdomyosarcoma
    • Osteosarcoma
    • Synovial sarcoma
    • Ewing sarcoma
    • Angiomyolipoma
    • Epithelioid angiomyolipoma
    • Leiomyoma
    • Haemangioma
    • Lymphangioma
    • Haemangioblastoma
    • Juxtaglomerular cell tumor
    • Renomedullary interstitial cell tumour
    • Schwannoma
    • Solitary fibrous tumor

Mixed epithelial and stromal tumour family
  • Adult cystic nephroma
  • Mixed epithelial and stroma tumour

Neuroendocrine tumors
  • Well differentiated neuroendocrine tumour
  • Large cell neuroendocrine carcinoma
  • Small cell neuroendocrine carcinoma
  • Paraganglioma

Renal haematopoietic neoplasms

Germ cell tumours

Metastatic tumours

Tumor-Like Lesions
  • Xanthogranulomatous pyelonephritis
  • IgG4 related disease

Described entities not currently in WHO
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