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Tumeur des cellules granuleuses adultes de l'ovaire

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IDDIS-GRANULOSA-CELL
TypeMaladie
Alias
AGCTTumeur des cellules granuleuses adultesOvaire du GCTTCG de type adultetumeur de cellules granuleusesДоросла гранульозоклітинна пухлина яєчника
Étatreviewed 2026-05-04 | pending_clinical_signoff
MaladiesAucune déclaration
SourcesSRC-ESMO-OVARIAN-2024 SRC-NCCN-OVARIAN-2025

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

Source YAML

icd_o_3_morphology8620/3
icd_o_3_topography["C56"]
icd_10C56
who_classificationWHO Classification of Female Genital Tumours 5th ed (2020): Adult granulosa cell tumour

Capacités

Notes

Adult GCT is defined molecularly by the FOXL2 p.C134W mutation (present in ~97% of cases), which is the only pathognomonic molecular alteration in any solid tumor. FOXL2 is a granulosa cell transcription factor; the C134W mutation is a dominant-negative gain-of-function that prevents normal granulosa cell differentiation. Clinically, tumors present with estrogen excess (abnormal uterine bleeding, endometrial pathology) or abdominal mass. Prognosis is generally favorable for stage I (90% 10-yr OS) but late recurrences (10–20 years) are characteristic. No FDA-approved targeted therapy as of 2026; FOXL2 inhibition and CDK4/6 inhibitors are in clinical trials. FOXL2 mutation testing is diagnostic, not therapeutic selection (ESCAT IIB — clinical utility as diagnostic/prognostic marker).

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Pertinence thérapeutique

Algorithmes

Maladie

Indication

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