Overall survival of 20-35%
Chemo responsive, but has a high relapse rate
Four components of treatment
- Induction - Aggressive multiagent chemotherapy (cyclophosphamide, topotecan, cisplatin, etoposide, doxorubicin, vincristine).
- Local Control - surgery and radiation.
- Consolidation - autologous bone marrow transplant.
- Biologic agents - isotretinoin (cis-RA)
Current COG high risk study is ANBL0532. To improve event free survival (EFS), this protocol uses:
- Dose intensification of the induction chemotherapy (with addition of dose-intensive topotecan and cyclophosphamide into induction)
- Tandem transplant using marrow support
- Escalation of radiotherapy dose for local control
