Ovarian Sertoli-Leydig cell tumours: How typical is their typical presentation?

Ovarian Sertoli-Leydig cell tumours (OSLCT) are rare and typically present with androgenic manifestations in women of the 2nd-3rd decade. Out of 228 diagnoses of ovarian sex cord-stromal tumours recorded at an academic institution during a 14-year period, eight women were surgically treated for OSLCT. Patient mean age was 54.8 years (range 19-81), five women being in the postmenopausal stage (62.5%). Only one woman presented with androgenic manifestations (12.5%), four with abnormal/postmenopausal uterine bleeding (50%), and three with abdominal pain (37.5%). Fertility sparing or radical surgery was performed depending on patient age and stage of disease. The only patient with an advanced disease (FIGO stage IV) was referred to palliative care postoperatively. The other seven were at FIGO stage I. Five of them were free from disease at a mean follow-up of 67 months, while the remaining two were lost at follow-up. The youngest woman of the series, treated with fertility-preserving unilateral salpingo-oophorectomy at the age of 19, had two spontaneous pregnancies and deliveries of healthy babies during a 10-year follow-up period. In conclusion, our single institution 14-year experience demonstrates that the diagnosis of OSLCT is particularly challenging since many patients are older than expected and lack androgenic manifestations. Impact statement • What is already known on this subjectOvarian Sertoli-Leydig cell tumours (OSLCT) are rare and are thought to typically present with androgenic manifestations in women of the 2nd-3rd decade. • What the results of this study addOur single institution 14-year experience shows that a high proportion of women with ovarian Sertoli-Leydig cell tumours may not present with androgenic manifestations, and many of them also are in the postmenopausal stage. Most patients have a good prognosis and fertility-preserving surgery in younger women can lead to spontaneous pregnancies and deliveries of healthy children after treatment. • What are the implications of these findings for clinical practice and/or further researchThe diagnosis of OSLCT is particularly challenging and therefore not reached before surgery in most of the cases. However, while hysterectomy with bilateral salpingo-oophorectomy and surgical staging are recommended for women with higher stage or no fertility wish, fertility-sparing surgery should be considered in younger women with early disease. Therefore, further research should focus on non-invasive diagnosis possibly by means of laboratory or imaging techniques.