90.2 ð åèçïŒé«åž«åè / PGY OSCEïŒ
90.2.0.1 ð Cram Sheet
90.2.0.1.1 ð¥ é« yield 10
- Clear cell 75% (VHL inactivation)
- Papillary Type 2 + FH (HLRCC): aggressive
- 70% incidentaloma (CT)
- Classical triad (flank pain + hematuria + mass) only 10%
- Paraneoplastic: PTHrP, EPO (erythrocytosis), renin (HTN), Stauffer
- T1 â partial nephrectomy preferred
- Adjuvant pembrolizumab high-risk post-nephrectomy (KEYNOTE-564)
- Metastatic 1st line IO+TKI: pembro+axi / nivo+cabo / pembro+len; IO+IO ipi+nivo
- Belzutifan (HIF-2α) for VHL (22E)
- Hereditary: VHL, MET (HRPC), FH (HLRCC), FLCN (BHD), TSC
90.2.0.1.2 ð¢ å¿ è
| Subtype | % | Genetics |
|---|---|---|
| Clear cell | 75% | VHL |
| Papillary Type 1 | 5% | MET activation |
| Papillary Type 2 | 5-10% | FH |
| Chromophobe | 5% | Multiple chr loss |
| é ç® | æžå |
|---|---|
| T1 | †7 cm |
| T1a | †4 cm |
| T1b | 4-7 cm |
| T2 | > 7 cm |
| T3 | Venous/perinephric |
| T4 | Beyond Gerota |
| Adjuvant pembro duration | 1 yr |
| IMDC risk groups | Favorable / intermediate / poor |
90.2.0.2 â é« yield
90.2.0.2.1 Hereditary RCC Syndromes
| Syndrome | Gene | Histology |
|---|---|---|
| VHL | VHL | Clear cell (multifocal, bilateral) |
| HRPC | MET (activating) | Papillary Type 1 |
| HLRCC | FH | Papillary Type 2 (aggressive) |
| Birt-Hogg-Dubé | FLCN | Chromophobe + oncocytoma + skin + lung |
| TSC | TSC1/2 | Angiomyolipoma + RCC |
| BAP1 | BAP1 | Multiple cancers (mesothelioma, RCC) |
| SDHB | SDHB | RCC + paraganglioma |
90.2.0.2.2 Drugs Quick
| Class | Drug |
|---|---|
| VEGFR TKI | Sunitinib, pazopanib, cabozantinib, lenvatinib, axitinib, tivozanib |
| Anti-PD-1 | Pembrolizumab, nivolumab |
| Anti-PD-L1 | Avelumab |
| Anti-CTLA-4 | Ipilimumab |
| mTOR | Everolimus, temsirolimus |
| Anti-VEGF | Bevacizumab |
| HIF-2α | Belzutifan (VHL) |
| IL-2 | Aldesleukin (high-dose; selected) |
90.2.0.3 ð¯ èªææª¢æž¬
- Clear cell %? â 75%
- Clear cell genetics? â VHL
- HLRCC gene? â FH (aggressive papillary 2)
- Classical triad %? â ~10%
- Paraneoplastic 4? â PTHrP, EPO, renin, Stauffer
- T1a vs T1b? â †4 cm vs 4-7 cm
- Localized T1 surgery? â Partial nephrectomy
- Adjuvant trial? â KEYNOTE-564 pembrolizumab à 1 yr
- Metastatic 1st line combos? â IO+TKI (pembro+axi/nivo+cabo/pembro+len) or IO+IO (ipi+nivo)
- Belzutifan target? â HIF-2α (VHL)
- VHL hereditary? â Hemangioblastoma + RCC + pheo + pancreatic NET
- Birt-Hogg-Dubé histology? â Chromophobe
- Stauffer syndrome? â Cholestasis without mets
- EPO from RCC? â Erythrocytosis paraneoplastic
- IMDC factors 6? â KPS, time, Hb, Ca, neutrophils, platelets
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