The World Health Organizatiοn (WHO) classification of tumours of the kidney is the most commonly used pathologic classification system for such disorders. The current revision, part of the 4th edition of the WHO series, was published in 2016 as part of the WHO Classification of Tumours of the Urinary System and Male Genital Organs 1,2. It is heavily based upon the International Society of Urological Pathology (ISUP) Vancouver classification of renal neoplasia of 2013 3 and supersedes the 2004 WHO classification. The 2016 version is reflected in the article below.
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Classification
Renal cell tumours
- 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 carcinoma
- succinate dehydrogenase-deficient renal 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 tumours
Nephroblastic and cystic tumours occurring mainly in children
- nephrogenic rests
- nephroblastoma
- cystic partially differentiated nephroblastoma
- paediatric cystic nephroma
Mesenchymal tumours occurring mainly in children
- clear cell sarcoma
- rhabdoid tumour
- congenital mesoblastic nephroma
- ossifying renal tumour of infancy
Mesenchymal tumours occurring mainly in adults
- leiomyosarcoma
- angiosarcoma
- rhabdomyosarcoma
- osteosarcoma
- synovial sarcoma
- Ewing sarcoma
- angiomyolipoma
- epithelioid angiomyolipoma
- leiomyoma
- haemangioma
- lymphangioma
- haemangioblastoma
- juxtaglomerular cell tumour
- renomedullary interstitial cell tumour
- schwannoma
- solitary fibrous tumour
Mixed epithelial and stromal tumour family
Neuroendocrine tumours
- well-differentiated neuroendocrine tumour
- large cell neuroendocrine carcinoma
- small cell neuroendocrine carcinoma
- phaeochromocytoma
Miscellaneous tumours
Metastatic tumours
Changes from prior versions
Several but not all familial forms of renal cell carcinoma have been subsumed into the corresponding histology, which can also arise sporadically (e.g. clear cell renal cell carcinoma in von Hippel-Lindau disease, chromophobe renal cell carcinoma in Birt-Hogg-Dubé (BHD) syndrome).
The entity previously known as multilocular cystic renal cell carcinoma or multilocular clear cell renal cell carcinoma is now described as multilocular cystic renal neoplasm of low malignant potential in recognition of the low incidence of recurrence and metastasis.
The size cutoff for papillary adenoma was increased from ≤0.5 cm to ≤1.5 cm.
Cystic nephroma has been divided into the adult and paediatric forms. The adult form is included along a spectrum of mixed epithelial and stromal tumours.
Renal carcinoids have been redesignated well-differentiated neuroendocrine tumours of the kidney and placed in a group with small cell neuroendocrine carcinomas, large cell neuroendocrine carcinomas, and paragangliomas (extra-adrenal phaeochromocytoma).
Several entities are newly established:
- hereditary leiomyomatosis and renal cell carcinoma-associated renal cell carcinoma
- succinate dehydrogenase-deficient renal carcinoma
- tubulocystic renal cell carcinoma
- acquired cystic disease-associated renal cell carcinoma
- clear cell papillary renal cell carcinoma
- paediatric cystic nephroma