Endocrine Abstracts

A 57-year-old gentleman presented with a very sudden change in hair colour to bright white and eyebrow loss. On further questioning, he had been shaving much less and had not been getting any erections. On examination he had gynaecomastia. Initial blood tests showed hypogonadotrophic hypogonadism (LH 0.6 IU/l, FSH 0.1 IU/l, testosterone 1.6 nmol/l) with an otherwise normal anterior pituitary hormonal profile. Oestradiol was found to be significantly elevated at 582 pmol/l. He was investigated for a possible testicular tumour which proved negative. He went on to have MRI of his adrenal glands which elucidated a large and suspicious mass in the right retroperitoneum. CT scanning confirmed this mass, of approximately 11 cm, was most likely to be adrenal in nature with concerns of invasion of the liver, right kidney, renal vein and inferior vena cava. FDG-PET showed that the adrenal lesion was FDG avid with no evidence of other sites of disease. A provisional diagnosis of adrenocortical carcinoma (ACC) ENSAT stage II was made. The adrenal MDT reviewed a scan from three years previously which showed a 10 mm right adrenal indeterminate lesion at that time. He underwent a right open adrenalectomy for removal of the adrenal lesion. The tumour was carefully dissected out without any spillage or capsular breach. Histology showed the tumour had a high Weiss score of 8 with a mitotic count of 10/50 HPF and Ki67 proliferation index was 20-25%. The lesion showed extra-adrenal extension with vascular and perineural invasion. Post-operative oestradiol levels normalised to 182 pmol/l, and testosterone recovered to 21 nmol/l. As the risk of recurrence was high, he completed six cycles of adjuvant EDP chemotherapy (etoposide, doxorubicin and cisplatin). He was also started on mitotane post-operatively which was uptitrated to 1.5 g twice daily plus prednisolone 4 mg daily. Nine months after surgery, his oestradiol began to increase again. On a CT thorax he was found to have pulmonary nodules and underwent VATS wedge resections. Histopathology confirmed metastatic ACC and genomic testing did not identify actionable mutations. Following MDT discussion, he was commenced on sunitinib and after three months of treatment, oestradiol remained elevated and a trial of letrozole was added. He went on to have radiofrequency ablation for liver metastases with subsequent gemcitabine and capecitabine chemotherapy after further disease progression. This case illustrates the challenges of managing a case of an aggressive oestrogen-secreting metastatic ACC.