Sex cord-stromal tumours, rare events in oncology necessitating multidisciplinary approach and referral pathways

Abstract

Tumours of the stroma (Leydig cells) and/or sex cords (Sertoli cells) represent approximately 8% of ovarian tumours and develop from the conjunctive tissue (respectively, interstitial and nurse cells) of the ovaries. Because these cells participate in ovarian hormonal function, most of the sex-cord or stromal tumours are able to secrete hormones (oestrogens, androgens, corticoids), which explains the hormonal dysfunctions associated with these cancers. Their prognoses are difficult to establish; some of the tumours are almost always benign (Sertoli cell tumours, Leydig cell tumours), whereas others are malignant but with variably delayed local-regional, or metastatic relapses. The histological criteria of aggressiveness are so poorly known that it is difficult to even propose a dichotomous, benign-malignant histopathological classification. If they do not present clinical criteria of 'malignancy', these tumours are considered to be of uncertain prognosis. In this group of tumours, the following might have 'malignant' behaviour: Granulosa cell tumours, androblastomas (or Sertoli-Leydig cell tumours), tumours of the sex cords with annealed tubules, tumours of the steroid-producing (theca) cells without any other specification, and fibrosarcomas. Surgery is the most important therapeutic modality and must be as conservative as possible to preserve reproductive function; it can be effectively combined with chemotherapy for advanced stages.