Endocrine Abstracts

Introduction: We report the first case of metastatic adrenocortical carcinoma (ACC) concomitantly secreting cortisol, aldosterone, catecholamines and androgens with the extensive distant metastasis at the time of diagnosis.

Case report: A 57-year-old woman referred for left adrenal mass (5.5×4.0 cm) two years after the diagnosis of breast cancer. The patient’s medical history revealed hypertension and diabetes mellitus. She has cushingoid and hyperandrogenemic appearance. Routine biochemical findings were normal, except for marked hypokalemia. Laboratory testing showed hyperaldosteronism, hyperandrogenism, hypercortisolism and increased urinary catecholamines; plasma renin: 170 pg/ml (2.71–16.51), plasma aldosteron: 678 pg/ml (29.4–161.5), basal ACTH: 7.8 pg/ml (0–46), cortisol: 27.2 μg/dl (5–25), DHEAS: 947 mg/dl (35–430), testosterone: 301 ng/dl (0–74), free testosterone: 8.23 pg/ml (0.29–3.18), androstenodion >8.6 ng/ml (0.3–3.7), 17 OH-progesterone: >20 ng/ml (0.08–1.3), urinary VMA: 16.6 mg/24 h (3.3–6.6) and urinary adrenalin: 43.8 μg/24 h (4–20). Loss of suppressibilility by both 2 and 8 mg dexamethasone and increased 24-h urinary free cortisol excretion (539 μg/24 h) suggested autonomous cortisol secretion. She uderwent left adrenalectomy and hepatic wedge resection for metastatic lession. Histopathologic diagnosis was reported as an adrenocortical carcinoma. Immunohistochemical examination showed positive staining of neoplastic cells for synaptophysin and inhibin, while tumor cells did not express S-100. Hepatic tumor histology was reported an ACC metastasis. Her laboratory results reached normal levels after operation. After the diagnosis of metastatic ACC, the patient received palliative chemotherapy, including carboplatin and etoposide. She was died 2 years after diagnosis.

Conclusion: ACC is a rare malignancy with a poor prognosis and presents with variable clinical pictures. Hormonally active adrenocortical carcinomas most commonly secrete cortisol while the co-secretion of multiple steroid hormones is rare. This is the first case of metastatis ACC in a patient with concurrent Cushing’s syndrome, Conn’s syndrome, hyperandrogenism and pheochromocytoma at the time of initial presentation.