Rare ovarian cancers (ROC) in France: Analysis of the first 100 patients (pts) prospectively included through an on-line observatory

16506 Background: A website (www.ovaire-rare.org) dedicated to germ cell and sex cord-stromal malignant tumors was developed in France to collect clinical cases and tumor samples of these uncommon ovarian cancers. The ultimate goal of this website is to better assess clinical and biological prognosis factors of ROC and to evaluate medical practice in the country. Methods: Adult pts with histological diagnosis of ROC in first-line or in first relapse were eligible. Results: From 03/2002 to 12/2007, 157 pts have been included and the first 100 were evaluated. Pts characteristics were: inclusion at diagnosis (78 pts) or in first relapse (22), histology: sex cord-stromal (61%), germ cell (30%), others (5%), median age 44 y. (range 18–79), median tumor size 12 cm (range 1–30), FIGO stage I 73%, II 10%, III 14%, IV 3%, ECOG PS 0 (88%). Review of histology by a panel was performed for 66 pts with discrepancy from initial diagnosis in 36%. Pts had initial conservative surgery in 56% and only 10% had lymph node dissection. 56 pts received BEP regimen (35 in first line, 13 in relapse). Grade 3–4 toxicity was observed in 26% of pts with 1 toxic death. Relapse occurred in 11/78 pts in first line and 5 died. OS at 5y was 94% and median PFS were 64 months (0–332). Independent prognostic factors for PFS were histology (other vs. germ cell or stromal; p=0.04), age (<60y vs. ≥60, p=0.03), stage (I/II vs. III/IV, p0.008), previous pregnancy (yes vs. no, p=0.04) and tumor size (<10 or >10cm, p=0.04). No patient in first line relapsed in optimal surgery subgroup compared to 16% for “other” surgery, p=NS. Conclusions: This on-line observatory on ROC offers an unique opportunity to better assess medical practice for these tumors in France. The histological discrepancies between initial diagnosis and second opinion confirm the importance to add a systematic slide review before treating ROC. The place of node dissection in these tumors with mostly a localized stage has to be better defined. Extension of the observatory to other rare gynecologic malignant tumor is planned. No significant financial relationships to disclose.