Endocrine Abstracts

Background: Steroid cell tumour of ovaries comes under sex cord stromal tumour that accounts less than 0.1% of all ovarian tumour. Majority are benign in childhood age group. It may produce steroids and testosterone resulting in virilisation and Cushing’s syndrome. Histology remains the gold standard for diagnosis of NOS. The gross appearance of NOS generally is well circumscribed, solid and noncalcifed with a lobulated appearance Till date only 10 cases has been reported in pubmed-search of steroid cell ovarian tumour NOS in childhood age group and this is first case report in Pakistan. We have also reviewed the literature on the epidemiology, clinical presentations, imaging and histological findings, and the treatment options on this disease presenting in prepubertal age group.

Case report: We present a 6 year old Pakistani girl who presented to us with complains of weight gain and hirsutism for past 10 months. On examination she had obvious cushingoid facies, facial hair growth, hypertrichosis, buffalo hump, acanthosis nigracans and marked hirsutism (ferriman Gallwey score of 25). Her blood pressure was >99th centile whereas tanner staging was P3 A1 B1. Investigation showed increased level of cortisol and loss of diurnal rhythm, increased testosterone levels and undetectable ACTH. B HCG and alpha fetoprotein levels were normal. Radiological it was a left adnexal mass of 6.9*8.1*4.9 cm (CC*TS*AP) measurement. She underwent left oophorectomy with findings of intact capsule and dilated vessels. Histopathological report revealed steroid cell tumour NOS (Not Otherwise specified) which was negative for malignancy. She was kept on intravenous hydrocortisone pre and post operative day to avoid adrenal crises. Postoperatively her levels of cortisol and testosterone became normal.

Conclusion: Steroid cell tumour NOS is a rare tumour which can be onerous to diagnose. Although rare, Steroid cell tumour of ovary should be considered in cases of childhood virilization. The typical clinical, radiological and histopathological findings can clinch the diagnosis in most of the cases however in difficult cases, immunohistochemistry can be useful.

Keywords: Steroid cell tumor, cushing syndrome, hyperandrogenism