Endocrine Abstracts

Estrogen-producing adrenal gland tumors (EPAGT) are extremely rare, accounting for only 1–2% of all adrenal tumors. They are most commonly observed in men or in children, and are unusual in women. They are almost always malignant even if they seem benign at presentation, and most of them have a poor prognosis. We present a case of a 71-year-old man with painful bilateral gynecomastia, without galactorrhea, over 4 months. Markedly elevated plasma estradiol levels were found (161.7 ng/l; normal 15.5–63.3) with low LH and FSH values. All other parameters were within normal range (b-HCG, PRL, TSH, FT4, total testosterone, SHBG, cortisol, ACTH, DHEA SO4, delta-4-androstenedione, 17OHPRG, fractionated metanephrines, cromogranine-A). Echographic images and mammography showed bilateral mammary gland hypertrophy excluding any nodules. CT abdominal scan indicated a large retroperitoneal mass measuring 14×8 cm with posterior and inferior deviation of the left kidney, with heterogeneus contrast uptake. It appeared to be a retroperitoneal tumor independent of any other retroperitoneal organ. To clarify its etiology, a biopsy was performed. The histology suggested an adrenal cortex tumor. It displayed cell proliferation with abnormal nuclei. Immunohystochemistry was positive for melan A, HMB45, INHA and focal AE1/AE3. The Ki-67 was around 15%. While it was impossible to distinguish between adenoma and carcinoma, the images were suggestive of malignancy. Although there were no signs of metastasis in toraco-abdomino-pelvic-CT, a PET-scan is arranged to exclude some other lesions. Gynecomastia in adult males is usually related with drugs and systemic or endocrine diseases. However, it is less often related with rare conditions, such as the testicular tumor of Leydig cells and the rarest adrenal feminizing tumor. Surgery is the main option for EPAGT, nevertheless the prognosis is very poor.