Adrenocortical carcinoma in a young adult male with chronic urticaria: A case report and literature review

BACKGROUND: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, often with an unfavorable prognosis. Radical adrenalectomy is the gold standard of treatment of localized disease. CASE DESCRIPTION: We report a case of a 23-year-old male patient who presented with persistent left flank pain and urticaria for 3 months. Imaging studies confirmed the presence of a large left adrenal mass with malignant features. The biochemical workup was unremarkable. Open left radical adrenalectomy was performed, the final pathologic examination showed ACC with negative surgical margins. The patient remained disease-free for eighteen months period of follow up after surgery. DISCUSSION: ACC is a rare neoplasm with poor prognosis and with an incidence of one in one million population. There is a slight female predilection. The ACC may be functional with a clinically pure endocrine syndrome like Cushing syndrome. Most of patients with ACC present with symptoms and signs of hormonal secretion. Adrenal computed tomography (CT) scanning and magnetic resonance imaging (MRI) are the imaging studies of choice in ACC. When feasible, total resection remains the treatment of choice for the definitive treatment of ACC. The benefit of the use of mitotane as an adjuvant treatment has been considered controversial. Adjuvant mitotane significantly decreases the recurrence and mortality rate after resection of ACC in patients without distant metastasis as proved by some studies, but these findings need further validation. CONCLUSION: ACC is a rare neoplasm characterized by a high risk of recurrence after surgical resection.