Low-grade glioma (LGG, WHO grade 2 — IDH-mutant)
Vista determinista de la entidad fuente YAML. La autoridad clínica sigue con los ID de fuente citados y estado de firma del revisor.
| ID | DIS-GLIOMA-LOW-GRADE |
|---|---|
| Tipo | Enfermedad |
| Aliaces | Astrocitoma difuso mutante IDHLGGGlioma de bajo gradoOligodendroglioma IDH-mutante 1p/19q-codeletadoГліома низького ступеня (WHO grade 2 — IDH-мутантна) |
| Situación | reviewed 2026-04-27 | pending_clinical_signoff |
| Enfermedades | Ninguna declarada |
| Fuentes | 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 CNS 5th ed (2021): Astrocytoma IDH-mutant grade 2 (9400/3) + Oligodendroglioma IDH-mutant 1p/19q-codeleted grade 2 (9450/3) |
Notas
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...
Utilizado por
Relevancia terapéutica
BMA-1P19Q-CODELETION-LGG- 1p/19q codeletion combined with IDH mutation defines oligodendroglioma (WHO CNS 2021 5th...BMA-IDH-MUTATION-GLIOMA-LOW-GRADE- IDH1/IDH2 mutation is the defining molecular feature of WHO grade 2 (and 3) diffuse gliom...BMA-MAP2K1-LGG- La activación de la vía MAPK es el sello distintivo del glioma de bajo grado (pLGG) y occ...
Algoritmos
ALGO-CASCADE-LFS-CARRIER-FAMILY-TESTING- ALGO-CASCADE-LFS-CARRIER-FAMILY-TESTINGALGO-GLIOMA-LGG-1L- ALGO-GLIOMA-LGG-1L
Enfermedad
DIS-ATRT- Atípico teratoide/tumor rabdoide (ATRT)DIS-MEDULLOBLASTOMA- Meduloblastoma
Indicación
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
Cuestionarios
QUEST-GLIOMA-LOW-GRADE-1L-STUB- Glioma de baja calidad, primera línea
Señal de alerta
RF-CMMRD-CONFIRMED-CARRIER- Variantes patogénicas / patogénicas probables biallélicas confirmadas en una reparación...RF-GLIOMA-LOW-GRADE-FRAILTY-AGE- Paciente de glioma de bajo grado con fragilidad: edad ≥75 con ≥2 comorbilidades + albumina <3,5 g/dL,...RF-GLIOMA-LOW-GRADE-HIGH-RISK-BIOLOGY- Low-grade glioma with high-risk molecular features: IDH-wildtype (per WHO 5th ed., reclas...RF-GLIOMA-LOW-GRADE-INFECTION-SCREENING- Infección activa o latente que requiere resolución / profilaxis antes de iniciar la PCV o te...RF-GLIOMA-LOW-GRADE-INTRACRANIAL-PRESSURE- Glioma de bajo grado con presión intracraneal elevada o convulsiones refractarias que requieren emerg...RF-GLIOMA-LOW-GRADE-ORGAN-DYSFUNCTION- Disfunción de los órganos basales que excluye estándar PCV (procarbazine / CCNU / vincristine), o...RF-GLIOMA-LOW-GRADE-TRANSFORMATION-PROGRESSION- Glioma de bajo grado con transformación en anaplásico (grado 3) o glioblastoma (grado 4): n...RF-NF1-CONFIRMED-CARRIER- Variante patógena/potencial patógena confirmada en NF1 O cumplimiento de NIH/Be...RF-NF1-FAMILY-HISTORY-SUSPICION- Patrón de pedigree familiar sugestivo de estado portador de la línea germinal NF1 en un ind...RF-NF2-SCHWANNOMATOSIS-CARRIER- Variante patógena confirmada de la línea germinal / patógena probable en NF2 (neurofibromina 2 / mer...RF-RHABDOID-PREDISPOSITION-CONFIRMED-CARRIER- El paciente tiene una variante patógena de gérmenes confirmada en SMARCB1 (cromosoma 22q11.2) o SM...
Fuentes
SRC-FOULKES-RHABDOID-2017- Vigilancia del cáncer en el síndrome de Gorlin y el síndrome de predisposición al tumor rabdoideSRC-TABORI-CMMRD-2017- Recomendaciones de Gestión Clínica y Vigilancia de Tumores de Reparación de Mismatch...