ESA-SRB-AOTA 2019

A Hairy Conundrum (#581)

Thora Y Chai 1 2 , Teresa Lam 1 3
  1. Department of Diabetes and Endocrinology, Westmead Hospital, Westmead, NSW, Australia
  2. Sydney Medical School, The University of Sydney, Sydney, NSW, Australia
  3. School of Medicine, University of New South Wales, Sydney, NSW, Australia

Introduction:

Androgen-secreting ovarian tumours are rare, accounting for <5% of ovarian neoplasms. We report a post-menopausal female with severe hyperandrogenisation from a Leydig cell tumour, identified after bilateral salping-oophorectomy and was undetected on multiple imaging scans.

 

Case: A 52-year-old female developed facial hirsutism six months after reaching menopause. Her hirsutism worsened six months later, with her face, chest and abdomen affected. New symptoms of virilisation also occurred (enhanced libido and voice deepening). She had a prior diagnosis of polycystic ovarian syndrome in her twenties and had never experienced hyperandogenisation.

 

She had elevated serum androgens (total testosterone level 7.4nmol/L (RR 0.4-1.4nmol/L); free androgen index (FAI) 26%; free testosterone level 196pmol/L (RR 4-21pmol/L)). Her DHEAS level was 1.4μmol/L (RR 1.3-6.2μmol/L) and her adrenal CT did not identify any adrenal lesions. Congenital adrenal hyperplasia was excluded with a 17-hydroxy progesterone level at 2.7nmol/L (RR < 1.3nmol/L) and a negative ACTH stimulation test. Cushing’s disease was excluded with a 24-hour urine free cortisol level at 183nmol/24hr (RR 100-330nmol/24hr) and a serum cortisol level at 40nmol/L (RR < 137nmol/L) after 1mg dexamethasone suppression. A transvaginal pelvic ultrasound did not identify any ovarian lesions or hyperthecosis.

 

Six months on, her plasma testosterone levels elevated to 11.5nmol/L and FAI was 35.9%. A CT chest/abdomen/pelvis and MRI abdomen did not identify any masses suggestive of androgen-secreting tumours. She was referred for a bilateral salping-oophrectomy, given ongoing suspicion of an androgen-secreting ovarian tumour. A right ovarian Leydig cell tumour was identified on histopathology with positive immunostaining for calretinin, inhibin and melan A (Ki-67 proliferation index <1%). Her serum androgens normalised post-surgery (total testosterone level 0.4nmol/L; free testosterone level 8.0pmol/L)

 

Conclusions: Leydig cell tumours frequently occur in post-menopausal women and should be suspected if sudden virilisation occurs. Imaging modalities do not always detect these tumours, making diagnosis a challenging affair.