Management of rare ovarian cancers: the experience of the French website "Observatory for rare malignant tumours of the ovaries" by the GINECO group: interim analysis of the first 100 patients.

BACKGROUND: Non-epithelial ovarian cancers are rare; their natural history is poorly understood and prognostic factors remain unclear. A French website (www.ovaire-rare.org) was developed to collect clinical cases and tumour samples in order to better define prognostic factors and develop specific trials. We report the results of the first 100 patients with germ cell (GCT) and sex cord-stromal (SCT) tumours.
METHODS: All adult patients with histological evidence of GCT or SCT at diagnosis or first relapse were eligible.
RESULTS: From 03/2002 to 06/2009, 180 patients were included; the first 100 were evaluated. Patient characteristics were: histology: SCT 61%, GCT 30%, others 5%; median age: 43 years; median tumour size: 12 cm; FIGO stages I-II: 83%, III-IV: 17%. Central pathology review (67 patients) differed from initial diagnosis in 37%. Fifty-six percent of the patients had initial conservative surgery and 10% lymph node dissection; 56 patients received chemotherapy. Eleven of the 78 first-line patients relapsed and 5 died; the 5-year OS rate was 94% and the median PFS 64 months.
CONCLUSIONS: This online observatory allows assessing medical practice for GCT and SCT in France. Histological discrepancies between diagnosis and second opinion confirm the need for systematic review before treatment. Extension to other rare gynaecologic malignancies is on-going.