We had a 29 year old lady with 11 years history of hirsutism and amenorrhoea. She was diagnosed as polycystic ovarian syndrome in a different hospital few years ago and commenced on metformin and spironolactone. She noticed worsening of hirsuitism over last year, needing to shave daily, along with loss scalp hair, deepening of voice and clitoromegaly. On examination, she was obese without any overt signs of Cushings syndrome. She had severe hirsuitism affecting face, arm, legs and abdomen. There was frontal balding with slightly masculine build. Her testosterone was high at 6.4 nmol/l and androstenidione were very high at 35 nmol/l. LH 5.6 U/l, FSH 4.0 U/l Oestradiol-200 pmol/l.DHES7.0 umol/l.
Beta-hcG-<2 IU/l. Alphafetoprotein -<2 kU/l. CT scan of the abdomen revealed 5.3 cm right ovarian mass with a normal appearing left ovary. There was no evidence of adrenal mass or any other pathology. She subsequently underwent bilateral oopherectomy and hysterectomy. Gross examination of the specimen showed a solid tumour with bright yellow central area. Histology revealed clear cell nests with occasional leydig cells. Immunohistochemistry showed the tumour to be vimentin, calretinin, CD99 and beta and alpha inhibin positive, but appears negative for S100, desmin, actin and EMA.This is in keeping with a diagnosis of steroid cell tumour with androgenic activity. No features of malignancy were identified. The biopsy of the left ovary was essentially normal. She had good resolution of hirsuitism and testosterone levels were normalised subsequently. She had longstanding history of amenorrhoea and hirsuitism with an onset in post-pubertal stage. This might have lead to the initial diagnosis of Polycystic ovarian syndrome. This case highlights the importance looking for evidence of virilization in hirsute women, even with longstanding symptoms. Androgens secreting steroid cell tumours are extremely rare ovarian tumours, especially in young women. These tumours generally tend to have good prognosis with less chance of recurrence.