Leber- und Gallenerkrankungen
Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.
| ID | DIS-HEPATOBLASTOMA |
|---|---|
| Art | Erkrankung |
| Aliase | Beckwith-Wiedemann-assoziiertes HepatoblastomLeber- und GallenerkrankungenKinder- und GallenzelltumorГепатобластома |
| Status | reviewed 2026-05-19 | pending_clinical_signoff |
| Erkrankungen | None declared |
| Quellen | SRC-AASLD-HCC-2023 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8970/3 |
|---|---|
| icd_o_3_topography | ["C22.0"] |
| icd_10 | C22.2 |
| who_classification | WHO-Digestive-System Tumore 5th ed (2019): Hepatoblastom |
Anmerkungen
Treatment philosophy combines pre-operative cisplatin-based chemo (SIOPEL PLADO / PLATO regimens) + surgical resection or liver transplantation. AFP is a key tumor marker — used for diagnosis, monitoring response, and surveillance in Beckwith-Wiedemann carriers (q3mo AFP from birth to age 3-4). High-risk subset (PRETEXT IV, +V involvement, metastatic) receives intensified regimens. Five-year EFS approaches ~70-80% across all risk groups in current SIOPEL / COG-AHEP cooperatives.
Verwendet von
Quellen
SRC-BRIOUDE-BECKWITH-WIEDEMANN-2018- Experten-Konsensdokument: Klinische und molekulare Diagnose, Screening und Management von...SRC-KALISH-BECKWITH-WIEDEMANN-2017- Surveillance Empfehlungen für Kinder mit Überwucherung Syndrome und...