Adult granulosa cell tumor of the ovary

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

ID	`DIS-GRANULOSA-CELL`
Type	Disease
Aliases	AGCTAdult granulosa cell tumorGCT ovaryadult-type GCTgranulosa cell tumorДоросла гранульозоклітинна пухлина яєчника
Status	reviewed 2026-05-04 \| pending_clinical_signoff
Diseases	None declared
Sources	`SRC-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_morphology	8620/3
icd_o_3_topography	["C56"]
icd_10	C56
who_classification	WHO Classification of Female Genital Tumours 5th ed (2020): Adult granulosa cell tumour

Capabilities

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).

Used By

Actionability

BMA-FOXL2-GRANULOSA-CELL - FOXL2 p.C134W (c.402C>G) is the defining molecular marker of adult granulosa cell tumor (...

Algorithms

ALGO-GRANULOSA-CELL-1L - ALGO-GRANULOSA-CELL-1L

Disease

DIS-SERTOLI-LEYDIG-OVARIAN - Sertoli-Leydig cell tumor of the ovary

Indication

IND-GRANULOSA-CELL-1L-CARBOPLATIN-PACLITAXEL - IND-GRANULOSA-CELL-1L-CARBOPLATIN-PACLITAXEL

Questionnaires

QUEST-GRANULOSA-CELL-1L-STUB - Adult granulosa cell tumor — first line