Adrenocortical Carcinoma Presenting With Concurrent Cushing Syndrome and Primary Hyperaldosteronism
Year: 2026
Type: article
Study design: Case Report
Abstract: Background/Objective Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy, with approximately half of cases demonstrating hormonal hypersecretion. Cortisol excess resulting in Cushing syndrome is the most common functional presentation. In contrast, aldosterone-producing ACC is distinctly uncommon, and concurrent secretion of both cortisol and aldosterone by a single tumor is rare. We describe a case of dual hormone–secreting ACC and review its diagnostic and therapeutic implications. Case Presentation A 64-year-old woman with long-standing treatment-resistant hypertension and poorly controlled type 2 diabetes mellitus presented with progressive weight gain, proximal muscle weakness, and worsening metabolic control. Biochemical evaluation demonstrated ACTH-independent hypercortisolism together with primary hyperaldosteronism. Computed tomography revealed a heterogeneous 4.3-cm left adrenal mass with indeterminate imaging characteristics suspicious for malignancy. Following preoperative endocrine optimization, including correction of hypokalemia and management of hypercortisolism, the patient underwent successful en bloc left adrenalectomy. Histopathologic examination confirmed adrenocortical carcinoma with a Weiss score of 6 and a Ki-67 proliferation index of approximately 25%, consistent with high-risk disease. Final staging was ENSAT stage II. Postoperatively, the patient experienced marked improvement in blood pressure control and glycemic parameters. Given high-risk pathologic features, adjuvant mitotane therapy was initiated. Discussion Dual cortisol- and aldosterone-secreting ACC is rare, with only a small number of cases reported in the literature. This phenotype likely reflects disordered steroidogenesis associated with underlying molecular abnormalities. Conclusion Comprehensive hormonal evaluation of adrenal masses is essential, as early recognition and multidisciplinary management of dual-secreting ACC can lead to improved clinical outcomes.
Source: AACE Endocrinology and Diabetes
Author: Naseem Eisa
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W7130394283