Pediatric adrenocortical carcinoma revealed by isolated Cushing syndrome: A case report

Abstract

Background: Adrenocortical carcinoma accounts for less than 0.2% of all pediatric malignancies. Virilization is the most common revealing sign, whereas hyperaldosteronism and Cushing syndrome are less common.We report a case of adrenocortical carcinoma which was revealed by Cushing syndrome and successfully treated in an adolescent girl. Case presentation: A female patient of 14 years old was admitted with complaints of progressive abdominal mass. Clinical findings included arterial hypertension (180/110 mmHg), Cushing syndrome, and a left flank mass approximately 10 cm in length. Biologic tests revealed hypercortisolism and hyperandrogenism. Abdominal CT showed a large, well-circumscribed, polylobed left adrenal mass. After contrast injection, there was a heterogeneous enhancement and demarcated areas of liquefaction without visible calcifications. A complete resection of the adrenal gland with lymphadenectomy was performed. The anatomopathological conclusion was a Weisse 6 adrenocortical carcinoma.The ENSAT classification for adrenocortical carcinoma in children was T1N1MO.Post-adjuvant chemotherapy was administered according to the etoposide/doxorubicin/cisplatin + mitotan (EDP-M) protocol in combination with hydrocortisone replacement therapy. Conclusion: Adrenocortical carcinoma is a rare childhood cancer. Cushing syndrome is a rare but potentially serious manifestation. Early treatment with surgical resection and chemotherapy improves the prognosis.

Keywords

• Pediatric adrenocortical carcinoma
• Cushing syndrome
• Surgery
• Case report