Niedriggradiges Gliom (LGG, WHO-Grad 2 — IDH-mutant)
Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.
| ID | DIS-GLIOMA-LOW-GRADE |
|---|---|
| Art | Erkrankung |
| Aliase | Diffuse Astrozytom IDH-mutantLGGNiedriggradiges GliomOligodendrogliom IDH-mutant 1p/19q-kodiertГліома низького ступеня (WHO grade 2 — IDH-мутантна) |
| Status | reviewed 2026-04-27 | pending_clinical_signoff |
| Erkrankungen | None declared |
| Quellen | SRC-EANO-GBM-2024 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 | 9400/3 |
|---|---|
| icd_o_3_topography | ["C71"] |
| icd_10 | C71 |
| who_classification | WHO-ZNS 5. ed (2021): Astrozytom IDH-mutante Grad 2 (9400/3) + Oligodendroglioma IDH -mutante 1p/19q-kodierte Grad 2 (9450/3) |
Anmerkungen
Defined molecularly, not just histologically: IDH-mutant grade 2 diffuse astrocytoma + IDH-mutant 1p/19q-codeleted oligodendroglioma. IDH-wild-type "diffuse astrocytomas" are reclassified as molecular GBM (DIS-GBM) per WHO 2021 even at grade 2 histology. Standard care: - Maximal safe surgical resection (extent of resection prognostic). - Low-risk (age <40 + gross-total resection): observation acceptable. - High-risk (age ≥40 OR subtotal resection): RT + adjuvant PCV (procarbazine + CCNU + vincristine; RTOG-9802 12-yr survival benefit) OR RT + temozolomide (CATNON for grade 3 anaplastic astrocytoma; equivalent for grade 2 in practice). Targeted therapy (paradigm-shifting 2024): - Vorasidenib (oral brain-penetrant pan-IDH1/2 inhibitor): FDA-approved Aug 2024 for IDH-mutant grade 2 astrocytoma / oligodendroglioma after surgery, in patients NOT requiring immediate RT/chemo. INDIGO trial: PFS HR 0.39 vs placebo; transformative for the watch-and-wait window. - Patient selection: residual / progressive non-enhancing disease after surgery; defers RT-induced cognitive toxicity. - Ivosidenib (IDH1-only) explored in earlier LGG trials but vorasidenib is preferred for CNS penetration. Long na...
Verwendet von
Therapeutische Relevanz
BMA-1P19Q-CODELETION-LGG- 1p/19q Codeletion kombiniert mit IDH-Mutation definiert Oligodendrogliom (WHO CNS 2021 5....BMA-IDH-MUTATION-GLIOMA-LOW-GRADE- IDH1/IDH2-Mutation ist das bestimmende molekulare Merkmal von WHO Grad 2 (und 3) diffuse Gliom...BMA-MAP2K1-LGG- MAPK-Wegaktivierung ist das Markenzeichen von pädiatrischem Low-Grade-Gliom (pLGG) und Okc...
Algorithmen
ALGO-CASCADE-LFS-CARRIER-FAMILY-TESTING- ALGO-CASCADE-LFS-CARRIER-FAMILY-TESTINGALGO-GLIOMA-LGG-1L- ALGO-GLIOMA-LGG-1L
Erkrankung
DIS-ATRT- Atypischer Teratoid/Rhabdoid-Tumor (ATRT)DIS-MEDULLOBLASTOMA- Medulloblastom
Anwendungsgebiet
IND-GLIOMA-LOW-GRADE-1L-RT-PCV- IND-GLIOMA-LOW-GRADE-1L-RT-PCVIND-NF1-CARRIER-PREVENTION-OBSERVATION- IND-NF1-CARRIER-PREVENTION-OBSERVATIONIND-NF1-CARRIER-PREVENTION-SURVEILLANCE- IND-NF1-CARRIER-PREVENTION-SURVEILLANCEIND-NF1-SUSPICION-PREVENTION-EMPIRICAL-SURVEILLANCE- IND-NF1-SUSPICION-PREVENTION-EMPIRICAL-SURVEILLANCEIND-NF1-SUSPICION-PREVENTION-TESTING- IND-NF1-SUSPICION-PREVENTION-TESTINGIND-NF2-CARRIER-INTENSIFIED- IND-NF2-CARRIER-INTENSIFIEDIND-NF2-CARRIER-SURVEILLANCE- IND-NF2-CARRIER-SURVEILLANCEIND-RHABDOID-PREDISPOSITION-CARRIER-INTENSIFIED- IND-RHABDOID-PREDISPOSITION-CARRIER-INTENSIFIEDIND-RHABDOID-PREDISPOSITION-CARRIER-SURVEILLANCE- IND-RHABDOID-PREDISPOSITION-CARRIER-SURVEILLANCE
Fragebogen
QUEST-GLIOMA-LOW-GRADE-1L-STUB- Niedriggradiges Gliom — erste Zeile
Warnhinweis
RF-CMMRD-CONFIRMED-CARRIER- Bestätigte biallelic keimlinien-pathogene / wahrscheinlich-pathogene Varianten in einer Fehlanpassung Reparatur...RF-GLIOMA-LOW-GRADE-FRAILTY-AGEAlter ≥ 75 Jahre mit ≥ 2 Komorbiditäten + Albumin < 3,5 g/dl,...RF-GLIOMA-LOW-GRADE-HIGH-RISK-BIOLOGY- Niedriggradiges Gliom mit hochrisikoreichen molekularen Eigenschaften: IDH-Wildtyp (pro WHO 5. ed., reklas...RF-GLIOMA-LOW-GRADE-INFECTION-SCREENING- Aktive oder latente Infektion erfordert Auflösung / Prophylaxe vor Beginn der PCV oder Te...RF-GLIOMA-LOW-GRADE-INTRACRANIAL-PRESSURE- Low-grade glioma with raised intracranial pressure or refractory seizures requiring emerg...RF-GLIOMA-LOW-GRADE-ORGAN-DYSFUNCTION- Ausgangsorgan Dysfunktion, die Standard vorschreibt PCV (procarbazine / CCNU / vincristine) oder...RF-GLIOMA-LOW-GRADE-TRANSFORMATION-PROGRESSION- Niedriggradiges Gliom mit Transformation zu anaplastischem (Grad 3) oder Glioblastom (Grad 4): n...RF-NF1-CONFIRMED-CARRIER- Bestätigte keimlinienpathogene / wahrscheinlich-pathogene Variante in NF1 ODER Erfüllung von NIH/Be...RF-NF1-FAMILY-HISTORY-SUSPICION- Familie Stammbaum Muster suggestive der Keimlinie NF1 Trägerzustand in einem asymptomatischen Indiv...RF-NF2-SCHWANNOMATOSIS-CARRIER- Bestätigte keimlinienpathogene / wahrscheinlich-pathogene Variante in NF2 (Neurofibromin 2 / merli...RF-RHABDOID-PREDISPOSITION-CONFIRMED-CARRIER- Patient hat eine bestätigte keimlinienpathogene Variante in SMARCB1 (Chromosom 22q11.2) oder SM...
Quellen
SRC-FOULKES-RHABDOID-2017- Krebsüberwachung im Gorlin-Syndrom und Rhabdoid-Tumor-Prädispositions-SyndromSRC-TABORI-CMMRD-2017- Klinisches Management und Tumor-Überwachung Empfehlungen von Inherited Mismatch Repair D...