Blastome de la pituitaire
Vue déterministe de l'entité source de la LJAM. L'autorité clinique demeure avec les ID de la source cités et l'état d'approbation de l ́examinateur.
| ID | DIS-PITUITARY-BLASTOMA |
|---|---|
| Type | Maladie |
| Alias | Blastome hypophysaire associé à DICER1Tumeur embryonnaire hypophysaire infantileПітуітарна бластома |
| État | reviewed 2026-05-20 | pending_clinical_signoff |
| Maladies | Aucune déclaration |
| 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 | 8273/3 |
|---|---|
| icd_o_3_topography | ["C75.1"] |
| icd_10 | C75.1 |
| who_classification | WHO Endocrine and Neuroendocrine Tumours 5th ed (2022): Pituitary blastoma — rare embryonal sellar / parasellar tumour of infancy, near-uniformly DICER1-driven |
Notes
Pituitary blastoma is the strongest single-tumour DICER1-syndrome signal — diagnosis in any infant should immediately trigger DICER1 germline testing AND cascade-testing of parents (>95% pituitary blastomas carry pathogenic germline DICER1). Distinct from far-more- common pituitary adenomas (which are not DICER1-driven and are typically adult-onset). Treatment: trans-sphenoidal or trans-cranial resection (technically challenging in infants — pituitary blastomas are bleeding-prone and locally invasive); adjuvant chemotherapy (carboplatin-VP16-cyclophosphamide-based regimens reported in case series); RT generally avoided under age 3 due to severe neurocognitive + endocrine toll. Hypercortisolism management (medical adrenal blockade, sometimes adrenalectomy) is an essential pre-operative bridge. Lifelong endocrine replacement (hydrocortisone, growth hormone, thyroid, gonadal axis) after curative-intent resection. Lifelong DICER1-syndrome surveillance for survivors.
Utilisé par
Maladie
DIS-CYSTIC-NEPHROMA- Néphrome kystiqueDIS-PINEOBLASTOMA- PineoblastomeDIS-PLEUROPULMONARY-BLASTOMA- Blastome pleuropulmonaire (PPB)DIS-SERTOLI-LEYDIG-OVARIAN- Tumeur des cellules Sertoli-Leydig de l'ovaire