Pineoblastoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-PINEOBLASTOMA |
|---|---|
| Type | Disease |
| Aliases | DICER1-associated pineoblastomaPNET of pineal regionPineal embryonal tumorПінеобластома |
| Status | reviewed 2026-05-20 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-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.
| icd_o_3_morphology | 9362/3 |
|---|---|
| icd_o_3_topography | ["C75.3"] |
| icd_10 | C75.3 |
| who_classification | WHO CNS Tumours 5th ed (2021): Pineoblastoma (Grade 4 embryonal CNS tumour); molecular subtypes include DICER1-altered, miRNA-processing-altered, MYC/FOXR2-activated, and RB1-altered groups |
Notes
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.
Used By
Disease
DIS-CYSTIC-NEPHROMA- Cystic nephromaDIS-PITUITARY-BLASTOMA- Pituitary blastomaDIS-PLEUROPULMONARY-BLASTOMA- Pleuropulmonary blastoma (PPB)DIS-SERTOLI-LEYDIG-OVARIAN- Sertoli-Leydig cell tumor of the ovary