Onco Wiki / Krankheit

Kieferoblastom

Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.

IDDIS-PINEOBLASTOMA
ArtErkrankung
Aliase
DICER1-assoziiertes PineoblastomPNET der KiefernregionKiefern-Embryonal-TumorПінеобластома
Statusreviewed 2026-05-20 | pending_clinical_signoff
ErkrankungenNone declared
QuellenSRC-COG SRC-NCCN-CNS-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Quelle: YAML

icd_o_3_morphology9362/3
icd_o_3_topography["C75.3"]
icd_10C75.3
who_classificationWHO-ZNS Tumore 5 ed (2021): Pineoblastom (Grad 4 embryonaler ZNS-Tumor); molekulare Subtypen umfassen DICER1-gealterte, miRNA-verarbeitende-alterte MYC/FOXR2-aktivierte und RB1-geänderte Gruppen

Fähigkeiten

Anmerkungen

Pineoblastoma is one of the canonical embryonal CNS manifestations of DICER1 syndrome — diagnosis in any child should trigger DICER1 germline testing regardless of family history. Distinct from pineocytoma (Grade 1, benign) and pineal parenchymal tumour of intermediate differentiation (PPTID, Grade 2-3). Treatment philosophy parallels other embryonal CNS tumours: maximal safe surgical resection (gross total resection is prognostically dominant); craniospinal irradiation in age-appropriate patients (deferred under age 3 due to neurocognitive toll); adjuvant high-dose chemotherapy regimens (often COG/SIOP protocols with carboplatin, vincristine, cyclophosphamide, cisplatin, etoposide combinations + autologous stem-cell rescue in high-risk cases). Trilateral retinoblastoma (bilateral RB1-carrier with pineal pineoblastoma) historically had near-uniform mortality; modern MRI surveillance from RB1-carrier diagnosis has shifted some cases to earlier detection with improved outcomes.

Verwendet von

Erkrankung