1、Biology and Therapeutic Vulnerabilities:Needs and OpportunitiesYing-Bei Chen,MD,PhDAttending PathologistDepartment of Pathology and Laboratory MedicineMemorial Sloan Kettering Cancer CenterVariant 4:UnclassifiedWHO Classification of Renal Cell Tumors(5th)Clear cell renal tumorsClear cell RCCMultiloc
2、ular cystic renal neoplasm-LMPPapillary renal tumorsPapillary adenomaPapillary RCCOncocytic and chromophobe renal tumorsOncocytoma Chromophobe RCCOther oncocytic tumors(emerging entities and NOS)Collecting duct tumorsCollecting duct carcinomaOther renal tumorsClear cell papillary renal cell tumorMuc
3、inous tubular and spindle cell carcinomaTubulocystic RCCAcquired cystic disease-associated RCCEosinophilic solid and cystic RCCRCC,NOS(unclassified)Molecularly defined RCCTFE3-rearrangedTFEB-alteredELOC(formerly TCEB1)-mutatedFumarate hydratase(FH)-deficientSuccinate dehydrogenase(SDHB)-deficientALK
4、-rearrangedSMARCB1-deficient renal medullary carcinoma(RMC)Tumors with papillary architecture Papillary RCC TFE3-rearranged RCC TFEB-altered RCC FH-deficient RCC ALK-rearranged RCC RCC,NOS(unclassified)Tumors with oncocytic/eosinophilic feature Chromophobe RCC SDHB-deficient RCC FH-deficient RCC TFE
5、B-altered RCC Tumors of Birt-Hogg-Dub Eosinophilic solid and cystic(ESC)RCC Other oncocytic tumorsEosinophilic vacuolated tumor(EVT)Low grade oncocytic tumor(LOT)Oncocytic neoplasm,NOS RCC,NOS(unclassified)Tumor with high-grade poorly differentiated morphology Clear cell RCC FH-deficient RCC SMARCB1
6、-deficient RMC Mucinous tubular and spindle cell carcinoma(MTSCC)with high grade transformation Collecting duct carcinoma RCC,NOS(unclassified)Unclassified:Unknown/Grey-zone Areas of ClassificationPapillary RCCNEJM 2016Cell Rep.2018TCGA-KIRP TFE3-rearranged RCC TFEB-altered RCC FH-deficient RCC ALK-