Ovarian steroid cell tumours are rare sex cord-stromal tumour with incidence of <0.1%(1 and 4). It’s subdivided based on cellular origin: stromal luteoma, Leydig cell tumour and steroid cell tumour (not otherwise specified, NOS)(2). The steroid cell tumour-NOS is the most common subtype and majority of these tumour secrete steroid hormones causing symptoms.
We present a case of 58-year-old post-menopausal female who presented with progressive hirsutism, deepening of voice, calf muscle hypertrophy, increased libido and clitoromegaly since Hysterectomy at age 48. She received Oral contraceptive pills and spironolactone for hirsutism.
Her other medical problems included new diagnosis of t2dm, dyslipidaemia, Class 1 Obesity and Polycystic ovarian syndrome.
Her biochemistry showed significant elevation in testosterone levels and underwent extensive workup to rule out neoplastic process. Imaging of her abdomen didn't show any ovarian or adrenal masses.
Tumour markers incl Ca 124, HE4 ca 19.9, CEA, free beta HCG were all normal. Prolactin 347 (<500miu/l). screening for Cushing, pheochromocytoma was negative, Inhibin B levels< 10 ng/L(10-20ng/L). GNRH stimulation test was inconclusive.
As she was post-menopausal, decision was made to proceed with bilateral salpingo-oophorectomy and histopathology showed right sided ovarian steroid cell tumour NOS (not otherwise specified) measuring about 2 cm. She had remarkable improvement in her symptoms and her testosterone levels normalised post-surgery.
Our case highlights the importance of investigations and workup to rule out neoplastic process when testosterone levels are significantly elevated even with the history of PCOS. Bilateral salpingo-oophorectomy can be safely offered as both diagnostic and therapeutic option to prevent metabolic complications in post-menopausal women even if imaging fails to detect ovarian lesion and other investigations completed to rule out adrenal cause. Steroid cell tumour does have malignant potential and can recur especially in post-menopausal women and needs regular follow up and is guided by histopathology results.