Delayed presentation of a virilising, pure testosterone-secreting adrenocortical carcinoma with coexistent composite myelolipoma and a venous thrombus extending to the heart.

A 40-year-old normotensive woman presented with abnormal facial hair for 4 years and amenorrhoea for 13 years. Hormonal, biochemical and haematological evaluation showed isolated elevation of serum testosterone and free testosterone. Her follicle-stimulating hormone and luteinising hormone were in the premenopausal range. Until recently she had reconciled to early 'menopause' and visited beauty clinics but never sought medical evaluation. Imaging revealed an enhancing left adrenal mass with fat densities and venous thrombus extending through the inferior vena cava to a 7 cm mass in the right atrium. She underwent left kidney-preserving surgery utilising hypothermic cardiopulmonary bypass with early clamping of the pulmonary artery without circulatory arrest. Histology showed adrenocortical carcinoma with composite incidental myelolipoma and neoplastic thrombus. At 2 months, testosterone has normalised and she is doing well. Isolated testosterone-secreting adrenocortical carcinoma with massive venous thrombus is rare as is coincidental composite macroscopic myelolipoma. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.