| Diagnostic work-up and treatment of AYA with medulloblastoma . | Current practice . | AYA considerations/uture directions and trials . |
|---|---|---|
| Diagnosis and subgrouping | Neuroradiology - Brain MRI pre and post (within 48 hours) surgery (Including: Axial or 3D T1-weighted, T2-weighted, FLAIR, DWI, and postcontrast T1-weighted sequences) - Spinal MRI should be done preoperatively or 10–14 days postoperatively Other diagnostics - CSF cytology: 10–14 days after surgery, if safe Pathology Histopathological diagnosis: classic, desmoplastic or nodular, extensive nodular, or large cell/anaplastic Molecularly defined subgroups: WNT-activated, SHH-activated and TP53WT, SHH-activated and TP53mut, or non-WNT and non-SHH. DNA methylation can aid in molecular subgrouping | Future directions: • Metabolic imaging and radiomics to predict molecular subgrouping • Liquid biopsy on CSF for disease monitoring |
| Surgical therapy | Maximal safe resection (midline transvermian or telovelar approach) | |
| Radiation therapy | Craniospinal irradiation (within 28–42 days after surgery) 36 Gy in daily fractions of 1.8 Gy, or a dose of 35.2 Gy in daily fractions of 1.6 Gy, each five times weekly + a local dose escalation to the posterior fossa with a total dose up to 54–55.8 Gy A craniospinal irradiation dose reduction to 23.4 Gy might be used in AYA with standard/average-risk disease | AYA considerations: • Role of proton radiation therapy • De-escalation of radiotherapy in select patients • Currently trialed in EORTC-1643-BTG/NOA-23 trial82 |
| Systemic therapy | Commonly used chemotherapy regimens include: 1. Packer chemotherapy regimen95 2. Cisplatin-etoposide-based combination90 | Tolerance is worse in adolescents and adults than in children. • Consider age and risk-dependent modulation of treatment91 • Consider decreased use of vincristine91 |
| Targeted therapy | SMO inhibitors at recurrence | Upfront use of SMO inhibitors in combination with chemoradiation • Currently trialed in EORTC-1643-BTG/NOA-23 trial82 |
| Diagnostic work-up and treatment of AYA with medulloblastoma . | Current practice . | AYA considerations/uture directions and trials . |
|---|---|---|
| Diagnosis and subgrouping | Neuroradiology - Brain MRI pre and post (within 48 hours) surgery (Including: Axial or 3D T1-weighted, T2-weighted, FLAIR, DWI, and postcontrast T1-weighted sequences) - Spinal MRI should be done preoperatively or 10–14 days postoperatively Other diagnostics - CSF cytology: 10–14 days after surgery, if safe Pathology Histopathological diagnosis: classic, desmoplastic or nodular, extensive nodular, or large cell/anaplastic Molecularly defined subgroups: WNT-activated, SHH-activated and TP53WT, SHH-activated and TP53mut, or non-WNT and non-SHH. DNA methylation can aid in molecular subgrouping | Future directions: • Metabolic imaging and radiomics to predict molecular subgrouping • Liquid biopsy on CSF for disease monitoring |
| Surgical therapy | Maximal safe resection (midline transvermian or telovelar approach) | |
| Radiation therapy | Craniospinal irradiation (within 28–42 days after surgery) 36 Gy in daily fractions of 1.8 Gy, or a dose of 35.2 Gy in daily fractions of 1.6 Gy, each five times weekly + a local dose escalation to the posterior fossa with a total dose up to 54–55.8 Gy A craniospinal irradiation dose reduction to 23.4 Gy might be used in AYA with standard/average-risk disease | AYA considerations: • Role of proton radiation therapy • De-escalation of radiotherapy in select patients • Currently trialed in EORTC-1643-BTG/NOA-23 trial82 |
| Systemic therapy | Commonly used chemotherapy regimens include: 1. Packer chemotherapy regimen95 2. Cisplatin-etoposide-based combination90 | Tolerance is worse in adolescents and adults than in children. • Consider age and risk-dependent modulation of treatment91 • Consider decreased use of vincristine91 |
| Targeted therapy | SMO inhibitors at recurrence | Upfront use of SMO inhibitors in combination with chemoradiation • Currently trialed in EORTC-1643-BTG/NOA-23 trial82 |
FLAIR, fluid-attenuated inversion recovery; DWI, diffusion-weighted imaging; CSF, cerebrospinal fluid.
| Diagnostic work-up and treatment of AYA with medulloblastoma . | Current practice . | AYA considerations/uture directions and trials . |
|---|---|---|
| Diagnosis and subgrouping | Neuroradiology - Brain MRI pre and post (within 48 hours) surgery (Including: Axial or 3D T1-weighted, T2-weighted, FLAIR, DWI, and postcontrast T1-weighted sequences) - Spinal MRI should be done preoperatively or 10–14 days postoperatively Other diagnostics - CSF cytology: 10–14 days after surgery, if safe Pathology Histopathological diagnosis: classic, desmoplastic or nodular, extensive nodular, or large cell/anaplastic Molecularly defined subgroups: WNT-activated, SHH-activated and TP53WT, SHH-activated and TP53mut, or non-WNT and non-SHH. DNA methylation can aid in molecular subgrouping | Future directions: • Metabolic imaging and radiomics to predict molecular subgrouping • Liquid biopsy on CSF for disease monitoring |
| Surgical therapy | Maximal safe resection (midline transvermian or telovelar approach) | |
| Radiation therapy | Craniospinal irradiation (within 28–42 days after surgery) 36 Gy in daily fractions of 1.8 Gy, or a dose of 35.2 Gy in daily fractions of 1.6 Gy, each five times weekly + a local dose escalation to the posterior fossa with a total dose up to 54–55.8 Gy A craniospinal irradiation dose reduction to 23.4 Gy might be used in AYA with standard/average-risk disease | AYA considerations: • Role of proton radiation therapy • De-escalation of radiotherapy in select patients • Currently trialed in EORTC-1643-BTG/NOA-23 trial82 |
| Systemic therapy | Commonly used chemotherapy regimens include: 1. Packer chemotherapy regimen95 2. Cisplatin-etoposide-based combination90 | Tolerance is worse in adolescents and adults than in children. • Consider age and risk-dependent modulation of treatment91 • Consider decreased use of vincristine91 |
| Targeted therapy | SMO inhibitors at recurrence | Upfront use of SMO inhibitors in combination with chemoradiation • Currently trialed in EORTC-1643-BTG/NOA-23 trial82 |
| Diagnostic work-up and treatment of AYA with medulloblastoma . | Current practice . | AYA considerations/uture directions and trials . |
|---|---|---|
| Diagnosis and subgrouping | Neuroradiology - Brain MRI pre and post (within 48 hours) surgery (Including: Axial or 3D T1-weighted, T2-weighted, FLAIR, DWI, and postcontrast T1-weighted sequences) - Spinal MRI should be done preoperatively or 10–14 days postoperatively Other diagnostics - CSF cytology: 10–14 days after surgery, if safe Pathology Histopathological diagnosis: classic, desmoplastic or nodular, extensive nodular, or large cell/anaplastic Molecularly defined subgroups: WNT-activated, SHH-activated and TP53WT, SHH-activated and TP53mut, or non-WNT and non-SHH. DNA methylation can aid in molecular subgrouping | Future directions: • Metabolic imaging and radiomics to predict molecular subgrouping • Liquid biopsy on CSF for disease monitoring |
| Surgical therapy | Maximal safe resection (midline transvermian or telovelar approach) | |
| Radiation therapy | Craniospinal irradiation (within 28–42 days after surgery) 36 Gy in daily fractions of 1.8 Gy, or a dose of 35.2 Gy in daily fractions of 1.6 Gy, each five times weekly + a local dose escalation to the posterior fossa with a total dose up to 54–55.8 Gy A craniospinal irradiation dose reduction to 23.4 Gy might be used in AYA with standard/average-risk disease | AYA considerations: • Role of proton radiation therapy • De-escalation of radiotherapy in select patients • Currently trialed in EORTC-1643-BTG/NOA-23 trial82 |
| Systemic therapy | Commonly used chemotherapy regimens include: 1. Packer chemotherapy regimen95 2. Cisplatin-etoposide-based combination90 | Tolerance is worse in adolescents and adults than in children. • Consider age and risk-dependent modulation of treatment91 • Consider decreased use of vincristine91 |
| Targeted therapy | SMO inhibitors at recurrence | Upfront use of SMO inhibitors in combination with chemoradiation • Currently trialed in EORTC-1643-BTG/NOA-23 trial82 |
FLAIR, fluid-attenuated inversion recovery; DWI, diffusion-weighted imaging; CSF, cerebrospinal fluid.
This PDF is available to Subscribers Only
View Article Abstract & Purchase OptionsFor full access to this pdf, sign in to an existing account, or purchase an annual subscription.
