Survival curves of molecularly defined adult granulosa cell tumor patients (MD-AGCT), AGCT FOXL2 wild-type (WT), and misdiagnosed other tumor types. A) Overall survival (OS) of the combined three AGCT (adult granulosa cell tumor) cohorts from three European centers. All outcome analyses were performed using Kaplan-Meier methods with log-rank (LR) testing and stratified log-rank (SLR) testing where cohorts were combined. All statistical tests were two-tailed. B) Disease-specific survival (DSS) of the combined three AGCT cohorts from three European centers. C) Overall survival after the first recurrence of MD-AGCT patients compared with patients diagnosed with misdiagnosed other types of cancers. Shown is only the patient cohort from Helsinki, Finland. Survival data is only after a patient’s first recurrence in the MD-AGCT group and the misdiagnosed other cancer types group. D) Overall survival for MD-AGCT from the Finnish population for patients who recurred and did not recur compared with an age-, sex-, and calendar year–matched Finnish population acquired as one-year intervals from the human mortality database (www.mortality.org). The green solid lines indicate MD-ACGTs with no observed survival event, the orange solid lines indicate MD-AGCTs with an observed survival event, and the red solid line indicates the expected survival of the general population. Ninety-five percent confidence bands are indicated by broken lines. Overlapping confidence bands indicates that there is no statistically significant difference in survival between those patients who recurred or did not recur compared with the survival of the matched population. E) Recommended diagnostic algorithm for accurate diagnosis of AGCT. Morphological assessment, FOXL2 immunohistochemistry, and FOXL2 mutation testing are applied in a step-wise fashion, as indicated, to identify MD-AGCTs, morphologically typical AGCT-WT (FOXL2 mutation negative), and other tumors (eg, sex cord stromal tumors, carcinomas, metastasis). AGCT = adult granulosa cell tumor; IHC = immunohistochemistry; MD-AGCT = molecularly defined adult granulosa cell tumor; WT = wild-type.