Functional Adrenocortical Carcinoma in Adolescence: A Case Report

Abstract

Introduction: Pediatric adrenocortical carcinoma is a rare and aggressive endocrine neoplasm, with an incidence of less than 0.5 cases per million children. Most are functional tumors, associated with endocrine manifestations such as Cushing's syndrome or virilization. Radical surgical treatment is the mainstay of therapy and the primary prognostic factor. Case report: We present the case of a 13-year-old male adolescent with headache, lower extremity edema, and severe hypertension. Physical examination revealed Cushingoid facies, central obesity, and pitting edema. Hormonal studies showed hypercortisolism and elevated DHEA-S. Contrast-enhanced computed tomography showed a heterogeneous left adrenal mass measuring 16.7 × 13.7 × 11.6 cm, with necrosis and calcifications, consistent with functional adrenocortical carcinoma. Complete surgical resection was performed via open laparotomy, achieving total excision of a 910 g mass, with areas of extensive necrosis and focal capsular rupture on histopathological analysis. Discussion: Early diagnosis of pediatric adrenocortical carcinoma remains a challenge due to its low frequency and variable clinical presentation. Tumors larger than 6 cm require an open approach to reduce the risk of capsular rupture and tumor dissemination. Factors such as extensive necrosis, large tumor size, and capsular rupture are associated with a worse prognosis and the need for adjuvant treatment. Conclusions: Functional adrenocortical carcinoma should be suspected in pediatric patients with Cushing's syndrome and large adrenal tumors. Complete oncologic surgical resection via laparotomy remains the treatment of choice and the main prognostic determinant.