Médulloblastome
Vue déterministe de l'entité source de la LJAM. L'autorité clinique demeure avec les ID de la source cités et l'état d'approbation de l ́examinateur.
| ID | DIS-MEDULLOBLASTOMA |
|---|---|
| Type | Maladie |
| Alias | PNET de cérebellum (terme historique)WHO grade IV embryonal CNS tumorМедулобластома |
| État | reviewed 2026-05-19 | pending_clinical_signoff |
| Maladies | Aucune déclaration |
| Sources | SRC-NCCN-CNS-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9470/3 |
|---|---|
| icd_o_3_topography | ["C71.6"] |
| icd_10 | C71.6 |
| who_classification | WHO CNS5 (2021): Medulloblastoma molecularly defined (WNT-activated, SHH-activated TP53-wt / TP53-mutant, non-WNT/non-SHH including Group 3, Group 4); histologically defined (classic, desmoplastic/nodular, MBEN, large cell/anaplastic) |
Notes
Molecular subgrouping has revolutionized risk stratification. WNT- activated MB has the most favorable prognosis and is candidate for treatment de-escalation in current SJMB12 / PNET5 protocols. SHH- activated TP53-mutant MB is high-risk and may warrant Li-Fraumeni workup + radiation avoidance considerations. Group 3 (MYC-amplified) is high-risk and intensified regimens are studied. Craniospinal RT in young children (<3 y) is generally avoided due to neurocognitive late effects — high-dose chemo + autologous stem cell transplant used as bridge. Surveillance after treatment: brain + spine MRI q3-6 mo years 1-2, q6-12 mo years 3-5, annually thereafter.
Utilisé par
Maladie
DIS-ATRT- Téréatoïde atypique/tume rhabdoïde (ATRT)