346.4 ð ç« æ«éèš Summary
346.4.1 ð äžå¥è©±çžœçµ
Urologic malignancies æ¶µèïŒ(1) renal cell carcinoma (RCC) â 90% adult kidney malignancyïŒclear cell most common (75%, VHL gene) + papillary (10-15%, MET type 1) + chromophobe (5%, BHD) + medullary (aggressive, sickle cell trait) + MiT family (young) + sarcomatoid (worst)ïŒRCC risk factorsïŒsmoking + obesity + HTN + CKD/dialysis + hereditary (VHL, HPRC, HLRCC, BHD, TSC)ïŒRCC presentationïŒoften incidental (50%) + classic triad rare (10%) + paraneoplastic syndromes (erythrocytosis EPO, hypercalcemia PTHrP, HTN renin, Stauffer hepatic dysfunction without mets, anemia)ïŒlocalized RCCïŒpartial nephrectomy preferred + adjuvant pembrolizumab (KEYNOTE-564 2021) for high-riskïŒmetastatic RCCïŒIO + TKI combinations practice-changing â pembrolizumab + lenvatinib (CLEAR, longest PFS), cabozantinib + nivolumab (CheckMate 9ER), pembrolizumab + axitinib (KEYNOTE-426), nivolumab + ipilimumab (CheckMate 214 intermediate-poor); belzutifan (HIF-2α inhibitor, LITESPARK-005, FDA 2021) for VHL RCC + select sporadicïŒ(2) bladder cancerïŒurothelial 90% > squamous (schistosomiasis) > adenocarcinomaïŒsmoking #1 risk + occupational (aromatic amines) + schistosoma + aristolochic acidïŒpainless gross hematuria 80% classicalïŒdiagnose with cystoscopy + cytology + CT urographyïŒNMIBC â TURBT + intravesical BCG (high-risk) / mitomycin (low-risk) / pembrolizumab (KEYNOTE-057 BCG-unresponsive)ïŒMIBC â radical cystectomy + neoadjuvant cisplatin chemo + adjuvant nivolumab (CheckMate 274)ïŒmetastatic 2024 paradigm-shiftïŒEV-302 enfortumab vedotin + pembrolizumab first-line (OS 31.5 vs 16.1 months chemo); cisplatin-based chemo + maintenance avelumab (JAVELIN Bladder 100) for cisplatin-eligible non-progressors; erdafitinib for FGFR3 alterations; sacituzumab govitecan (Trop-2 ADC)ïŒ(3) upper tract urothelial carcinoma (UTUC): Lynch syndrome + aristolochic acid; nephroureterectomyã
346.4.2 ð æ²»ç粟èŠ
- localized RCCïŒpartial nephrectomy (preferred) > radical nephrectomy; ablation for elderly small tumors; adjuvant pembrolizumab (KEYNOTE-564) high-risk
- metastatic RCC first-line IO+TKI combosïŒpembrolizumab + lenvatinib (CLEAR â best PFS) OR cabozantinib + nivolumab (CheckMate 9ER) OR pembrolizumab + axitinib (KEYNOTE-426) OR nivolumab + ipilimumab (CheckMate 214 â intermediate/poor risk)
- VHL-associated RCCïŒbelzutifan (HIF-2α inhibitor, FDA 2021, LITESPARK-005)
- NMIBCïŒTURBT + intravesical BCG (high-risk, gold standard) or mitomycin (low-risk single dose); pembrolizumab (KEYNOTE-057) for BCG-unresponsive CIS
- MIBCïŒneoadjuvant cisplatin (GC, dose-dense MVAC) + radical cystectomy + LN dissection + adjuvant nivolumab (CheckMate 274); bladder preservation tri-modality alternative (TURBT + chemoradiation)
- metastatic urothelial 2024ïŒEV-302 enfortumab vedotin (Nectin-4 ADC) + pembrolizumab first-line (OS 31.5 vs 16.1 mo chemo) â practice-changing
- cisplatin-eligible advanced urothelialïŒGC or MVAC chemo + maintenance avelumab (JAVELIN Bladder 100)
- FGFR3-altered urothelialïŒerdafitinib (Balversa, FDA 2019)
- second-line metastatic urothelialïŒenfortumab vedotin, sacituzumab govitecan
- UTUCïŒnephroureterectomy (gold standard) ± adjuvant chemo (POUT trial)
346.4.3 ð¯ ç§é«åž«çèåæé
- RCC clear cell 75% + VHL gene mutationïŒmost common subtype + most amenable to targeted + IO therapyïŒpapillary 10-15% (MET in type 1), chromophobe 5% (BHD), medullary aggressive (sickle cell trait association)
- RCC paraneoplastic syndromes ç¶å žïŒerythrocytosis (EPO secretion), hypercalcemia (PTHrP), HTN (renin), Cushing (ACTH ectopic), Stauffer syndrome (hepatic dysfunction without metastases â IL-6 driven, resolves with nephrectomy), anemia
- RCC classic triad (flank pain + hematuria + abdominal mass) only 10% â late stage; 50% incidental detection on imaging for other reasons
- metastatic RCC IO+TKI combinations standard 2018-2024ïŒCLEAR (pembrolizumab + lenvatinib â longest PFS 23.9 mo) + CheckMate 9ER (cabozantinib + nivolumab) + KEYNOTE-426 (pembrolizumab + axitinib) + CheckMate 214 (nivolumab + ipilimumab for intermediate/poor risk)
- adjuvant pembrolizumab RCC KEYNOTE-564 (2021) â FDA approved for high-risk after surgery
- belzutifan (Welireg) HIF-2α inhibitorïŒVHL-associated RCC FDA 2021 + LITESPARK-005 (2023) for sporadic RCC second-line > everolimus
- bladder cancer urothelial 90% + painless gross hematuria 80% classical + smoking #1 risk factor + occupational (aromatic amines: dye, rubber, leather, textile)
- schistosoma haematobium â squamous cell bladder carcinomaïŒEgypt, Africa endemic; chronic inflammation drives squamous transformation
- NMIBC intravesical BCGïŒgold standard for high-risk + CIS; pembrolizumab (KEYNOTE-057) for BCG-unresponsive alternative to cystectomy
- EV-302 (2024) paradigm-shifting for metastatic urothelialïŒenfortumab vedotin (Nectin-4 ADC) + pembrolizumab first-line doubles OS (31.5 vs 16.1 months chemo) â new standard