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

Abstract

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 epithelial neoplasms. Objectives: The purpose of our study was to report the clinical and pathological findings in young patients with these tumours in our population. Material and Methods: The present observational cross-sectional study included all subjects <21 years of age diagnosed with ovarian SCST, in Aga Khan University Hospital Histopathology Laboratory, Karachi, Pakistan, from January 1992 till July 2013. Results: Of the total of 513 SCSTs presented during the study period, 39 fulfilled inclusion criteria and were assessed. The age range was 4-250 months. Most of the tumours presented at stage-1 and an abdominal mass was the most common presenting symptom, 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 fibrothecomas spectrum, 2 Sertoli leydig cell tumours (SLCT) and one a sex cord tumour with annular tubules (SCTAT). Detailed immunohistochemical analyses were performed in 33 cases. Recurrence/metastasis was noted in 4/21 cases with follow-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, although surprisingly sclerosing stromal tumours were also common. Clinical symptoms due to hormone secretion in premenstrual girls and menstrual disturbance in menstruating girls are common presenting features.

Ovarian sex cord stromal tumors: an institutional experience

“…This is in accordance with the available international literature. [6][7][8][9] The histomorphological observations in our study are similar to other reports. 6,7 We observed that most of the tumors were unilateral, ranging from 5 to 15 cm in size.…”

“…We encountered one case of sex cord tumor with annular tubules. The percentage of Juvenile Granulosa cell tumors was more than that observed by Cartault et al and Scheneider et al while similar to those reported by Haroon et al 4,9,10 However, sertoli leydig cell tumors was found to be less frequent than fibrothecoma group as compared to those in other studies. 6,7 This could be due to reluctance of patients with symptoms of virilization to come for treatment due to social stigma attached.…”

Ovarian Cancer in Children and Adolescents: Treatment and Reproductive Outcomes

“…In contrast, we found only one from 48 cases (2.1%) which had sex cord-stromal tumor, whereas the report by Brookfield et al showed 5.7% (Brookfield et al, 2009) and another study by Hassan et al showed 12.3% of 57 cases (Hassan et al, 1999). In Asia, Haroon et al from Pakistan reported 39 cases of ovarian sex cord-stromal tumor in children and young girls from their 20 year-experience (Haroon et al, 2014). These discrepancies may derive from the limitation of our data and the large number of patients in other studies may be deviated from the percentages of outcomes.…”

Genetic Polymorphisms of TCF7L2 Lack Influence on Risk of the Polycystic Ovary Syndrome - a Systemic Analysis

“…Polycystic ovary syndrome (PCOS) is a proliferative disorder among women of reproductive age, and currently considered a predominantly hyperandrogenic syndrome (Azziz et al, 2009;Bilici et al, 2013;, Haroon et al, 2014;Liu et al, 2013;Takiar et al, 2014a;2014b). The three main diagnostic guidelines are the 1990 National Institutes of Health (NIH) criteria (Dunaif, 1992) T2DM is a disease characterized by impaired insulin sensitivity.…”