96.2 ð åèçïŒé«åž«åè / PGY OSCEïŒ
96.2.0.1 ð Cram Sheet
96.2.0.1.1 ð¥ é« yield 10
- Liposarcoma most common adult STS
- GIST KIT+/PDGFRA+ = imatinib paradigm
- Osteosarcoma 15-25 yr metaphysis: MAP chemo + surgery
- Ewing: EWS-FLI1 t(11;22); VAC/IE
- Sarcoma loves lung mets
- Li-Fraumeni (TP53) osteosarcoma + STS
- NF1 â MPNST
- Synovial sarcoma: SS18-SSX
- Bone mets sources: breast, prostate, lung, RCC, thyroid, MM
- Bone modifying: zoledronic / denosumab / radium-223 (prostate)
96.2.0.1.2 ð¢ å¿ è
| Tumor | Genetics |
|---|---|
| Osteosarcoma | TP53 (Li-Fraumeni), RB1 |
| Ewing | EWS-FLI1 t(11;22) |
| Synovial | SS18-SSX |
| GIST | KIT+ / PDGFRA+ |
| Chordoma | EZH2 |
| MPNST | NF1 loss |
| Rhabdomyosarcoma | PAX3-FOXO1 (alveolar) |
| Angiosarcoma | KDR |
| é ç® | æžå |
|---|---|
| Osteosarcoma age | 15-25 yr |
| Ewing age | Adolescent |
| Imatinib GIST | 400-800 mg/d |
| Adjuvant imatinib | 3 yr (high-risk) |
96.2.0.2 â é« yield
96.2.0.2.1 GIST Treatment Ladder
- Imatinib 400 mg
- Imatinib 800 mg (KIT exon 9)
- Sunitinib (post-imatinib)
- Regorafenib
- Ripretinib
- Avapritinib (PDGFRA D842V â only)
96.2.0.2.2 Sarcoma Drugs Quick
| Drug | Use |
|---|---|
| Doxorubicin | Backbone STS |
| AIM (doxorubicin + ifosfamide + mesna) | High-grade STS |
| Pazopanib | Refractory STS |
| Trabectedin | Post-anthracycline STS |
| Eribulin | Liposarcoma + leiomyosarcoma post-anth |
| Larotrectinib | NTRK fusion |
| Tazemetostat | EZH2 / INI1-loss |
| Ipilimumab + nivolumab | Alveolar soft part |
| Imatinib | GIST |
| MAP (high-dose MTX + doxorubicin + cisplatin) | Osteosarcoma |
| VAC/IE | Ewing |
96.2.0.2.3 Bone Mets Treatment
- Bone-modifying agents:
- Zoledronic acid (q3-4 wk for cancer)
- Denosumab (anti-RANK-L; q4 wk for cancer)
- Radium-223 (alpha-emitter; prostate bone-only mets)
- RT for pain (single 8 Gy effective)
- Prophylactic fixation if Mirels score ⥠9
- Treat underlying cancer
- SCC emergent (Ch 80)
96.2.0.3 ð¯ èªææª¢æž¬
- Most common adult STS? â Liposarcoma
- GIST genetics? â KIT+ / PDGFRA+
- GIST 1st? â Imatinib
- PDGFRA D842V drug? â Avapritinib
- Osteosarcoma age? â 15-25 yr
- Osteosarcoma chemo? â MAP
- Ewing fusion? â EWS-FLI1 t(11;22)
- Ewing chemo? â VAC/IE
- Sarcoma mets favorite? â Lung
- Li-Fraumeni gene? â TP53
- NF1 â ? â MPNST
- NTRK fusion drug? â Larotrectinib
- Bone modifying agents? â Zoledronic / denosumab / radium-223
- RT pain dose? â Single 8 Gy
- Mirels ⥠9? â Prophylactic fixation
â ïž AI èçš¿ã