Key content

• Nonepithelial ovarian cancers (NEOCs) are rare forms of ovarian cancer, including malignant ovarian germ cell tumours (MOGCTs), sex cord stromal tumours (SCSTs) and ovarian sarcoma.

• Tumour markers including alpha-fetoprotein, human chorionic gonadotrophin, lactate dehydrogenase and inhibin can be useful in aiding preoperative diagnosis of malignant ovarian masses.

• NEOCs are staged using a similar classification to epithelial ovarian tumours; however, the staging system for male germ cell tumours is considered a more useful prognostic tool.

• These tumours can occur at any age; however, many present in the reproductive years, and fertility-sparing treatments are often required.

• Advanced-stage MOGCTs and SCSTs are managed with debulking surgery and platinum-based adjuvant chemotherapy, with the regimen of bleomycin, etoposide and cisplatin being most widely used.

Learning objectives

• To understand the classification and pathology of NEOCs.

• To describe the role of tumour markers in diagnostic criteria and to be aware of the FIGO staging classifications and their prognostic use.

• To understand management protocols and follow-up regimens.

Ethical issues

• Patients of reproductive age risk infertility if preoperative diagnosis is incorrect and more extensive surgery involving bilateral salpingo-oophorectomy is performed.