Ovarian Sex Cord Stromal Tumours in Children and Young Girls - A More Than Two Decade Clinicopathological Experience in a Developing Country, Pakistan

Background: Ovarian sex-cord stromal tumours (SCST) are rare, and relatively infrequent in children.These have to be distinguished from more common germ cell tumors in children and also from benign epithelialneoplasms.
Objectives: The purpose of our study was to report the clinical and pathological findings in youngpatients with these tumours in our population. Material and
Methods: The present observational cross-sectionalstudy included all subjects <21 years of age diagnosed with ovarian SCST, in Aga Khan University HospitalHistopathology Laboratory, Karachi, Pakistan, from January 1992 till July 2013.
Results: Of the total of 513SCSTs presented during the study period, 39 fulfilled inclusion criteria and were assessed. The age range was4-250 months. Most of the tumours presented at stage-1 and an abdominal mass was the most common presentingsymptom, along with menstrual disturbance. The left side ovary was slightly more affected (53.5%). Of the total,15 were juvenile granulosa cell tumours (JGCT), 11 sclerosing stromal tumours (SST), 10 of the fibrothecomasspectrum, 2 Sertoli leydig cell tumours (SLCT) and one a sex cord tumour with annular tubules (SCTAT). Detailedimmunohistochemical analyses were performed in 33 cases. Recurrence/metastasis was noted in 4/21 cases withfollow-up data.
Conclusions: Ovarian sex cord stromal tumours are very rare in young age in our population,and usually present at an early stage. Most common among these are juvenile granulosa cell tumours, althoughsurprisingly sclerosing stromal tumours were also common. Clinical symptoms due to hormone secretion inpremenstrual girls and menstrual disturbance in menstruating girls are common presenting features.