ABSTRACT
This chapter focuses on rare gynaecological cancers which represent an extraordinarily high number of the gynaecological cancers. The Gynaecological Cancer Intergroup published their rare tumour guidelines in late 2014 in a supplement to the International Journal of Gynaecological Cancer. There are well-established guidelines which include the rarer tumours, specifically by European Society of Medical Oncology which covers non-epithelial cancers, and by the National Comprehensive Cancer Network and in a recently published textbook on rare gynaecological cancers. Post-menopausal bleeding associated with endometrial hyperplasia and a solid adnexal mass may raise suspicion of a functioning sex cord tumour. These patients should be investigated and assessed in the standard manner and staging and histological diagnosis is the key points in the management of these tumours. For post-menopausal women with ovarian and uterine suspected tumours, the standard of care will usually include total hysterectomy, bilateral salpingo-oophorectomy and consideration of omentectomy and nodal dissection.
