Retinoblastom
Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.
| ID | DIS-RETINOBLASTOMA |
|---|---|
| Art | Erkrankung |
| Aliase | Erbliches RetinoblastomKinderretinoblastomRBSporadic RetinoblastomTrilaterales RetinoblastomРетинобластома |
| Status | reviewed 2026-05-18 | pending_clinical_signoff |
| Erkrankungen | None declared |
| Quellen | SRC-COG SRC-NCCN-PEDIATRIC-SARCOMA |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9510/3 |
|---|---|
| icd_o_3_topography | ["C69.2"] |
| icd_10 | C69.2 |
| who_classification | WHO-Augentumore 5 ed (2024): Retinoblastom |
Anmerkungen
Most common pediatric ocular malignancy. Germline RB1 testing mandatory at diagnosis — drives sibling cascade screening, lifetime second-primary surveillance (osteosarcoma, soft-tissue sarcomas, melanoma; ~50% lifetime risk of second cancer in germline carriers), and treatment choice (avoid external-beam RT in germline carriers because of post-RT sarcoma risk in field). Treatment: intra-arterial melphalan chemo (Group A-B-C eyes) is modern standard with high eye-salvage rates; enucleation for Group D-E or RT-induced cataract / glaucoma; systemic chemo (carboplatin + etoposide + vincristine) for bilateral / disseminated disease.
Verwendet von
Therapeutische Relevanz
BMA-RB1-GERMLINE-RETINOBLASTOMA- RB1 keimlinienpathogene Varianten verursachen vererbbares Retinoblastom (~40% des Retinoblastoms c...
Erkrankung
DIS-PINEOBLASTOMA- Pineoblastom