CNS Tumors

Question 1

Treatment of GBM with no targetable mutations: 1st Line treatment

Answer 1

Primary treatment: Surgical Resection
Adjuvant Therapy:
RT + concurrent TMZ 75 mg/m2 followed by adjuvant TMZ ± 150-200 mg/m2 tumor treating fields for 6 months

Question 2

Treatment of subependymal giant cell astrocytoma in patients with tuberous sclerosis

Answer 2

Everolimus - driven by mTOR pathway

Question 3

Treatment of GBM with no targetable mutations: Timeline of Progression and 2nd line treatment options

Answer 3

Within the first 3 months after completion of RT and concomitant TMZ, diagnosis of recurrence can be indistinguishable from pseudoprogression on neuroimaging

Second Line Treatments: no SOC, PS >2
- Surgical re-resection if possible or re-radiation
- Bevacizumab (only improvement in PFS)
- TMZ (esp if MGMT mutant)
- Lomustine or carmustine
- PCV (Procarbazine, lomustine and vincristine)
- Regorafenib

Question 4

Post- Resection Management of Ependymoma:
- All grades
- Grade 1
- Grade 2
- Grade 3

Answer 4

• All: CSF Analysis (2 weeks after surgery), Brain & Spinal MRI
• Grade 1: surveillance alone
• Grade 2 and Grade 3: Standard involved field RT
• Craniospinal RT only indicated if CSF involvement

Question 5

Clinical Criteria and Post-Resection Treatment of high risk medulloblastoma

Answer 5

High Risk Criteria:
- Residual tumor > 1.5 cm or unresectable tumor
- Disseminated disease within or outside of the neuroaxis
- Large cell medulloblastoma

Treatment Post-Resection: HIGH RISK
Craniospinal radiation with systemic therapy followed by q post-radiation systemic therapy
- Systemic therapy with RT: Cisplatin, lomustine, and vincristine
- Systemic therapy post- RT: Cisplatin, cyclophosphamide, and vincristine

Question 6

Post-Resection Treatment of Oligodendroma
- All
- Grade 1
- Grade 2
- Grade 3

Answer 6

All: Brain + Spinal MRI, CSF Analysis
Grade 1 : Surveillance
Grade 2 + 3: Involved field RT

Question 7

Clinical Criteria and Post-Resection Treatment of standard risk medulloblastoma

Answer 7

Standard Risk:
- No evidence of metastasis
(brain, spine, CSF, extraneural)
- Small-volume residual disease (< 1.5 cm)
- Classic or desmoplastic histology

Treatment Post-Resection:
- Standard field RT
- Reduced-dose craniospinal RT with systemic therapy followed by post-radiation systemic therapy

Question 8

Treatment of Recurrent Medulloblastoma:
A. If prior systemic therapy (4)
B. If achieve CR with conventional doses of chemotherapy
C. If SHH mutations and prior systemic therapy

Answer 8

A. If prior systemic therapy
- high-dose cyclophosphamide ± etoposide
- Oral etoposide
- TMZ
- TMZ/irinotecan/bevacizumab
B. If achieve a CR with conventional doses of systemic therapy
- High-dose systemic therapy with ASC reinfusion
C. If SHH pathway mutations after prior systemic therapy: Vismodegib

Question 8

Treatment of IDH mutant Grade 3 Astrocytoma

Answer 8

1. Resection
2. Adjuvant RT followed by temzolomide for 6 months