Gynaecomastia is common, occurring in 3050% of healthy men, and its aetiology is usually benign. Clinical features inclusive of recent onset, rapid progression, and loss of libido and muscle strength, indicate the need to exclude a malignant aetiology.
A 48-year-old man presented with an 18-month history of such clinical features. Investigations revealed cortisol 1136 nmol/l, 17-hydroxyprogesterone 6.6 nmol/l, androstenedione 13.3 nmol/l, DHEAS 21.8 μmol/l, urinary free cortisol >1380 nmol/24 h, testosterone 2.8 nmol/l, oestradiol 1261 pmol/l, FSH 0.1 U/l, LH 0.1 U/l, prolactin 598 μIU/ml, SHBG 51.3 nmol/l, fT4 11.9 pmol/l, fT3 2.9 pmol/l, TSH 1.6 mU/l, AFP <6 IU/ml and HCG <1 mIU/ml. Ultrasound of testes was normal but a CT scan demonstrated a 17×13×17.5 cm left adrenal mass, multiple liver metastases, extensive para-aortic lymphodenopathy and lung metastases. The impression formed was one of an oestradiol secreting adrenal carcinoma. At laparotomy a 1.6 kg left adrenal tumour was excised with as much nodal disease as could be safely removed. Histology confirmed adrenocortical carcinoma.
Adrenocortical carcinoma is a rare disease with an annual incidence of 12 per million. Feminising oestradiol secreting tumours account for <1% of cases. Peripheral conversion of high levels of androstenedione secreted by the tumour to oestradiol is thought to contribute to the high oestradiol levels. The ENSAT (European Network for the Study of Adrenal Tumours 2008) stage 4 (T4, N1, M1) disease this patient was found to have confers a very poor prognosis. Despite chemotherapy with mitotane, cisplatin, doxorubicin and etoposide, he died 8 months after presentation.