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. 2023 Jan 16;115(1):8–22. doi: 10.32074/1591-951X-818

Table I.

Summary of most important changes in the WHO 2022 classification of kidney tumours.

Renal Tumour Entity Key changes in WHO 2022
Papillary renal cell carcinoma (PRCC) Subclassification into type 1 and type 2 PRCC no longer recommended Morphologic spectrum of PRCC expanded to include the following patterns: biphasic PRCC, papillary renal neoplasm with reverse nuclear polarity, and Warthin-like PRCC
Clear cell papillary renal cell tumour Name change from “carcinoma” to “tumour” owing to benign behavior
TFE3-rearranged RCC and TFEB-altered RCC Previously considered together as “MIT family of RCCs”. Now separated into two distinct types: TFE3-rearranged RCC and TFEB-altered RCC (that includes TFEB-rearranged RCC and TFEB-amplified RCC).
Fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) FH-deficient RCC is the preferred name over hereditary leiomyomatosis associated RCC
SMARCB1 (INI1)-deficient renal medullary carcinoma Name change from former “medullary carcinoma”
Eosinophilic solid and cystic renal cell carcinoma (ESC RCC) New entity (included under “Other renal tumours”)
Anaplastic lymphoma kinase-rearranged renal cell carcinoma (ALK-rearranged RCC) New entity (included under “Molecularly defined renal carcinomas”)
ELOC (formerly TCEB1)-mutated RCC New entity (included under “Molecularly defined renal carcinomas”)
Low-grade oncocytic tumour (LOT) Emerging entity (included under “Other oncocytic tumours of the kidney”)
Eosinophilic vacuolated tumour (EVT) Emerging entity (included under “Other oncocytic tumours of the kidney”)
Oncocytic renal neoplasms of low malignant potential NOS Suggested name for eosinophilic/oncocytic tumours with borderline features between oncocytoma and chromophobe RCC that do not fit into any specific entity. This term should be used for a group of heterogeneous sporadic, eosinophilic/oncocytic tumours with borderline features (included under “Other oncocytic tumours of the kidney”). “Hybrid oncocytic tumors” is a suggested term for eosinophilic/oncocytic tumors with borderline features that occur in a hereditary setting, such as Birt-Hogg- Dubé syndrome.