A Rare Case of Aldosterone-Producing Adrenocortical Carcinoma with Co-Secretion of Cortisol and Estradiol

Presentation Number: SAT 382
Date of Presentation: April 1st, 2017

Karen Domingo Lazaro* and Perie Adorable-Wagan
The Medical City, Pasig City, Philippines

Abstract

Background: Adrenocortical carcinoma is a rare malignancy with an incidence of approximately one to two per million population per year. 62% of adrenocortical carcinomas present as functioning tumors with the most common presentation being Cushing’s syndrome or a combination of hypercortisolism and virilization. Aldosterone producing adrenocortical carcinoma is even more uncommon, comprising 0% to 7% of all functioning adrenocortical carcinomas. We report a rare case of adrenocortical carcinoma with a clinical picture of primary aldosteronism and subclinical Cushing’s syndrome. Clinical case: An 18-year old male presented with uncontrolled hypertension, recurrent bilateral leg weakness and hypokalemia. He had no signs of hypercortisolism. Laboratory tests showed elevated plasma aldosterone concentration (47.66 ng/dL, normal 4.20 - 20.15), suppressed plasma renin activity (0.04 ng/ml/hr, normal 0.30-1.90) and elevated aldosterone/renin ratio (1,191.5). Adrenal CT scan showed a 4.7 x 4.1 x 4.8 cm left adrenal mass with enhancement features suggestive of a lipid-poor adenoma. Complete hormonal evaluation showed elevated 24 hour urine free cortisol (131.20 ug/24 hours, normal 20.00 - 90.00), nonsupressible cortisol after 1 mg dexamethasone (2.8 ug/dL, normal < 1.8), normal basal cortisol (10.00 ug/dL), normal basal ACTH (0.25 pg/ml, normal 10 - 90), elevated estradiol (60.00 pg/ml, normal 11 - 44), normal DHEA-S (176.10 ug/dL, normal 45.10 - 385), elevated 24 hour urine metanephrine (6.8 mg/24 hours, normal < 1.0) and normal plasma free metanephrine (5.650 pg/ml, normal < 90.00). Laparoscopic adrenalectomy was done with steroid coverage. Histopathologic diagnosis was malignant adrenal neoplasm (adrenocortical carcinoma versus pheochromocytoma) based on the Weiss criteria. Immunohistomorphology supported the diagnosis of adrenocortical caricnoma. The Ki67 index, a marker of proliferative activity was 5-10%. No further treatment such as chemotherapy was done after complete surgical resection. His blood pressure decreased and aldosterone, cortisol and estradiol levels returned to normal. Conclusion: Aldosterone producing adrenocortical carcinoma is a rare malignancy. In the work up of suspected adrenal carcinoma, complete hormonal evaluation is necessary even if clinical symptoms are absent. The pattern of tumor secretion and tumor characteristics on CT scan may point to the malignant potential of the tumor. It is important to exclude hypercortisolism and pheochromocytoma as these require pre operative preparation. Complete surgical resection is the cornerstone of treatment and Ki67 index is the most powerful prognostic marker and used to guide treatment decisions. Long-term monitoring is recommended with imaging and hormonal evaluation used as tumor markers for recurrence.

 

Nothing to Disclose: KDL, PA