An unusual case of Steroid cell tumour of the ovary — ASN Events

An unusual case of Steroid cell tumour of the ovary (#336)

Ni Ni Khin 1 , Louise CH Ciin 1
  1. The Endocrinology Department, The Royal Darwin Hospital, Tiwi, NT 0800, Australia

Background: Ovarian steroid cell tumours account for 0.1% of all ovarian tumours.1 There are three categories depending on their cellular origins; 20% are stromal cell tumours, 20% Leydig cell, and 60% not otherwise specified (NOS).1, 2 94% are unilateral and 28.6% are malignant.2 Post menopausal virilization due to these tumours may be autonomous or gonadotropin dependent.4 ,5 In animal studies, there is no association between immunosuppression and development of ovarian tumours.3

Case: A 53-year old lady with renal transplant presented with a 5-year history of male pattern hair loss and hirsutism. There was no clitoromegaly or other features of virilization. BMI was 29 without features of Cushing’s.

Past history: Renal transplant from living brother at aged 26, CIN II, hypertension and osteopaenia.

Medications: azathioprine 100 mg, prednisolone 3 mg daily, perindopril/hydrochlorothiazide 5/1.25 mg and atorvastatin 20 mg daily. Her cyclosporine was ceased a few months post-transplant due to side effects.

She had menarche at 13 with regular periods. She conceived first child at 23 without difficulty, second child 6 years after transplant and ten miscarriages in between. She became menopausal at 32. She has never been on HRT.

Investigations: testosterone 7.5 & 10 (0.3-2.6 nmol/L) on 2 occasions, free testosterone 136, SHBG 37, androstenedione 18 (3-10 nmol/L), dehydroepiandrosterone-sulphate 2.3 (1-11 umol/L), FSH 46 IU/L, LH 29 IU/L, prolactin 6.3 (3-19 ug/L).
CT revealed normal adrenals, numerous punctuate calcification in left ovary without definitive right ovarian tissue.
Ovarian venous sampling showed elevated left ovarian testosterone. (table)
 

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She underwent bilateral salpingo-oopherectomy. Histology revealed ovarian steroid cell tumour (NOS), and Leydig cell hyperplasia. Post-operative testosterone was 0.5 nmol/L. She regained scalp hair.

Conclusion: Our post-menopausal lady on immunosuppressives developed gradual onset of virilizaton. She was confirmed to have ovarian steroid cell tumour (NOS) which may be autonomous or gonadotrophin dependent. Association between immunosuppression and development of ovarian tumours has not been reported so far.

  1. Young R.H., Scully R.E. Sex cord-stromal, steroid cell, and other ovarian tumours with endocrine, paraendocrine, and paraneoplastic manifestations. In Blaustein’s Pathology of the Female Genital Tract Edited by: Kurman RJ. New York, Springer-Verlag: 1994; 783-847.
  2. Scullt R.E., Young R.H., Clement P.B. Steroid cell tumours. In Tumours of the Ovary, Mal-developed Gonads, Fallopian Tube, & Broad Ligament. Washington, DC: Armed Forces Institute of Pathology: 1996; 227-38.
  3. Sorianello E., Schillaci R., Chamson-Reig A., Lux-Lantos V. and Libertun C. Actions of Immunosuppressor Drugs on the Development of an Experimental Ovarian tumour. Experimental Biology and Medicine. 2002; 227:658-664.
  4. Lamberts, S.W.J., Timmers, J.M., Oosterom, R., Verleun, T., Rommerts, F.G. and De Jong, F.H. Testosterone secretion by cultured arrhenoblastoma cells: Suppression by luteinizing hormone releasing hormone agonist. J. Clin. Endocrinol. 1982; 54, 450–454.
  5. Picon, M.J., Lara, J.I., Sarasa, J.L., Recases, J.D., Clouet, R., Gonzalo, M.A. and Rovira, A. Use of long–acting gonadotrophin-releasing hormone analogue in a postmenopausal woman with hyperandrogenism due to a hilus call tumour. Eur. J. Endocrinol. 2000; 142, 619–622.
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