Onco Wiki / Krankheit

Brustblättriges Blastom

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

IDDIS-PITUITARY-BLASTOMA
ArtErkrankung
Aliase
DICER1-assoziiertes HypophysenblastomInfantiler Hypophyse-EmbryonaltumorПітуітарна бластома
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_morphology8273/3
icd_o_3_topography["C75.1"]
icd_10C75.1
who_classificationWHO Endokrine und Neuroendokrine Tumore 5 ed (2022): Hypophysisches Blastom — seltener embryonaler Sellar/Parasellar-Tumor der Kindheit, nahezu einheitlich DICER1-getrieben

Fähigkeiten

Anmerkungen

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.

Verwendet von

Erkrankung