Endocrine Abstracts

Introduction: Malignant feminizing adrenal tumors are very rare occurring generally in men. The lack of gynecomastia is even rarer in those tumor secreting estrogens alone, or with other adrenal hormones especially cortisol as in the following case.

Case report: A male aged 44, consulted in May 2012 for abdominal pain. Radiological assessment discovered a large tumor measuring 120×95 mm located in the left adrenal with numerous metastases (pulmonary, retroperitoneal and parietal). He was operated on without any hormonal assessment. The pathological examination confirmed the adrenal origin with a Weiss’s score =5. Six months later the tumor relapsed and he was operated on again, and then sent to our department for hormonal exploration. Clinical examination showed a skinny man with severe fatigue and anorexia. No sign of Cushing’s syndrome was present. There is not any gynecomastia or galactorrhea. Penile size and testicle volume were normal, body heart growth and repartition were normal too. Many parietal nodules were present in the thoracic and abdomen areas. Biological assessment showed high plasma cortisol which failed to be suppressed by dexamethasone (respectively 784.34 nmol/l (50–550) and 4530, high estradiol (E₂)=645.32 pmol/l (<50), high 17OH progesterone=11.7 nmol/l, but low testosterone: 6 nmol/l (10–41)). Radiological exploration showed numerous metastases in the pulmonary, retroperitoneal, and abdominal areas. He died in September 2013 after chemotherapy failure.

Conclusion: Feminizing adrenal tumors are very rare. Gynecomastia which is a classical manifestation may be absent as in this case where metastases were already present at diagnosis. The rapid evolution, severe protein degradation, and breast resistance to E₂ maybe some reasons for the missing gynecomastia.