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Blostoma de pituitaria

Vista determinista de la entidad fuente YAML. La autoridad clínica sigue con los ID de fuente citados y estado de firma del revisor.

IDDIS-PITUITARY-BLASTOMA
TipoEnfermedad
Aliaces
Blostoma pituitario asociado a DICER1Tumor embrionario hipofisario infantilПітуітарна бластома
Situaciónreviewed 2026-05-20 | pending_clinical_signoff
EnfermedadesNinguna declarada
FuentesSRC-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.

Fuente YAML

icd_o_3_morphology8273/3
icd_o_3_topography["C75.1"]
icd_10C75.1
who_classificationWHO Endocrine and Neuroendocrine Tumours 5th ed (2022): Pituitary blastoma — rare embryonal sellar / parasellar tumour of infancy, near-uniformly DICER1-driven

Capacidades

Notas

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.

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