Introduction/Background Granulosa cell tumors account for approximatively 3% of ovarian tumors. The juvenile histological type (JGCT) represents 5% of them, and remains relatively unknown. Our aim was to describe clinicopathologic characteristics and to evaluate prognostic factors.
Methodology We retrospectively studied medical records of 40 patients referred to our center for JGCT between November 2010 and 2018.
Results Thirty-six patients had confirmed JGCT. The mean age was 19,9 years (range 2,5 months - 47,4 years). Abdominal pain (53%), vaginal bleeding (31%), palpable mass (28%), were the most common presenting symptoms. The distribution of FIGO stage was: 16 patients at stage Ia, 17 Ic, 1 IIa, 1 IIIb, 1 IIIc. Thirty-two patients (89%) had conservative surgery. Sixteen (44%) received adjuvant chemotherapy (Ic=13, IIa=1, IIIb=1, IIIc=1), mostly bleomycine/etoposide/cisplatine regimen. The mean follow up was 35 months (range 1 to 204 months). Nine patients recurred (Ia=1, Ic=6, IIa=1, IIIc=1) with a mean time of 12,1 months (range 1–47). Among them, 4 had a cystectomy at the initial surgery: 3 without adjuvant chemotherapy, 2 died of disease; 4 stage Ic did not received adjuvant chemotherapy neither. Three patients died after a mean of 21,2 months (range 9,5–32,4). At 36 months: progression free survival was 70,4% [54,9–90,2], overall survival was 85,6% [71,4–100]. The main prognosis factor of recurrence was advanced FIGO stage (p< 0,001). Age >15 years (p= 0,09)and cytonuclear atypia (p= 0,08)tended to be associated to relapse too. Intra operative tumor rupture (p= 0,03) was a significant risk factor of death.We observed 5 pregnancies with term deliveries.
Conclusion The main prognostic factors are: FIGO stage and intra operative tumor rupture. Fertility sparing surgery is safe for stage Ia, reasonable for stage Ic combined with adjuvant chemotherapy. Patients with cystectomy had poor outcome; unilateral salpingo-oophorectomy must be done sytematically.
Disclosure Nothing to disclose.
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