Endocrine Abstracts

JOINT1854

Adrenal cortical carcinoma is a rare malignant tumor of the adrenal cortex and represents 0.5-2% cases per million per year with peaks of occurrence in the first decade between 40 and 50 years of age. The main circumstances of discovery are hormonal hypersecretion in 40%-70% of cases and tumor syndrome in 40%-60% of cases. Hyperestrogenism is revealed in men by gynecomastia or a drop in libido with testicular involution. We report the case of a 57-year-old patient, with no particular history, admitted for treatment of a left adrenocortical tumor discovered by bilateral gynecomastia evolving for 6 months. The clinical examination reveals a patient in altered general condition (weight loss, profound asthenia, anorexia), bilateral gynecomastia present for 6 months, decreased libido and abdominal pain. There are no signs of hyperadrenocorticism or palpable abdominal mass. The hormonal assessment shows an increase in the precursors of adrenal androgens 17OHP, D4androstendione, SDHEA. Major hyperestrogenism is found and there is no biological hypercortisolism. Thoraco-abdomino-pelvic CT scan revealed a large left adrenal tumor mass with a malignant appearance suggestive of corticoadrenaloma with hepatic metastases invading the left renal vein and the infrahepatic IVC. A biopsy of the adrenal mass performed externally came back in favor of a left adrenocorticaloma with positive immunohistochemistry, synaptophysin and Melan A. The evolution in the hospital environment was noted by a hypercalcemia probably paraneoplastic, the initiation of bisphosphonates was deferred given the altered general condition of the patient who died following a state of hemorrhagic shock. Feminizing adrenocortical tumors or estrogen-secreting adrenal tumors are extremely rare. They are mainly observed in men and children in whom the main symptoms are breast enlargement. Their diagnosis is mainly based on the assessment of estrogens. A mixed secretion attested by the elevation of adrenal precursors and a large tumor argue in favor of malignancy. Treatment should be based on surgery associated with op’DDD or aromatase inhibitors with or without standard or targeted chemotherapy. The prognosis depends on the stage of the tumor at the time of diagnosis and the course of the disease, but in general the final prognosis is extremely poor, especially in adults in whom benign tumors are exceptional.