CNS Tumors Flashcards
Treatment of GBM with no targetable mutations: 1st Line treatment
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
Treatment of subependymal giant cell astrocytoma in patients with tuberous sclerosis
Everolimus - driven by mTOR pathway
Treatment of GBM with no targetable mutations: Timeline of Progression and 2nd line treatment options
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
Post- Resection Management of Ependymoma:
- All grades
- Grade 1
- Grade 2
- Grade 3
- 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
Clinical Criteria and Post-Resection Treatment of high risk medulloblastoma
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
Post-Resection Treatment of Oligodendroma: grade 1 vs grade 2
Grade 2 tumors
– For patients who undergo complete or near-complete resection of a grade 2 oligodendroglioma, watchful waiting and treatment with the IDH inhibitor, vorasidenib.
- This is an area of uncertainty, and decisions should be individualized based on shared decision-making. Either approach is reasonable depending on patient preferences.
- For most patients with residual disease after surgery and no uncontrolled disease-related symptoms, we suggest vorasidenib rather than radiation therapy (RT) plus chemotherapy or watchful waiting (Grade 2C).
- Postoperative therapy with RT and chemotherapy is advised for most patients with uncontrolled disease-related symptoms.
•Grade 3 tumors
– Postoperative therapy with RT and chemotherapy is indicated in most patients with newly diagnosed grade 3 (anaplastic) oligodendroglioma, regardless of the degree of resection or other risk factors.
- When patients with grade 2 or 3 oligodendroglioma are selected for RT, we recommend RT plus chemotherapy rather than RT alone (Grade 1A). This recommendation is based on the survival benefit conferred by combination therapy in long-term follow-up of trials in both grade 2 and grade 3 oligodendroglial tumors.
Clinical Criteria and Post-Resection Treatment of standard risk medulloblastoma
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
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
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
Treatment of IDH mutant Grade 3 Astrocytoma
- Resection
- Adjuvant RT followed by temzolomide for 6 months
