Recurrent Somatic DICER1 Mutations in Nonepithelial Ovarian Cancers — NEJM

Management of rare ovarian cancers: The experience of the French website «Observatory for rare malignant tumours of the ovaries» by the GINECO group

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.

 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.

free full text: Non-epithelial ovarian cancer: ESMO Clinical Recommendations for diagnosis, treatment and follow-up

Note: the papers from ESMO (ovarian cancer) requires registration/access is free 1) Classification of germ cell ovarian tumors 2) Classification of sex cord stromal ovarian tumors "This set of recommendations applies to invasive epithelial ovarian carcinoma; the management of tumors of low malignant potential (‘borderline’) is not covered here."

Nonepithelial ovarian cancer: Outcomes after aggressive treatment with surgery and platinum-based chemotherapy

Conclusions:
In this high-recurrence-risk group of nonepithelial ovarian cancers, aggressive treatment with surgery and platinum- based chemotherapy resulted in excellent survival outcomes.