Background: Cushing syndrome is a clinical syndrome which comprises symptoms and signs reflecting excessive circulating glucocorticoid concentrations. It can be classified into: adrenocorticotrophic hormone (ACTH)-independent and ACTH-dependent. This incidence is approximately 2 to 5 new cases per million people per year. Only approximately 10% of the new cases each year occur in children. In children under 7 years, unilateral (single) adrenal tumors presenting with Cushing syndrome are often malignant. The prognosis Adrenal cancer associated with Cushing syndrome has an extremely poor.
Objective: To report a case of cushing syndrome et causa adrenocortical carcinoma in an 11-year and 6-month old boy.
Case: An 11-year and 6-month old boy was admitted to the hospital with pneumonia, abdominal enlargement, hirsutism, and weakness in both of legs. On examination, height was 133 cm (< 5th percentiles) and weight was 40 kg (BMI/U 85th-95th percentiles). There was: hypertension, hirsutism, double chin, buffalo hump, sentripetal obesity, striae rubrae and ecchymosis. Tanner stage was G3, P3, diameter of testis 3x2x1,5 cm. A marked increase of serum cortisol, hypokalemia, hyperglycemia, MSCT scan abdomen with constrast suggest left suprarenal mass (± 8,9x7,8x9,9 cm) with bone age delayed. It’s confirmed the diagnosis of ACTH independent Cushing syndrome. The patient underwent adrenalectomy sinistra with an immediate cortisol serum decline in the postoperative phase. Histology confirmed the diagnosis of adrenocortical carcinoma. Glucocorticoid replacement therapy was instituted. The patient was planned to chemotherapy.
Conclusion: It is a full blown case of clinically ACTH independent Cushing syndrome caused by adrenocortical carcinoma. The management of patients requires a multidisciplinary approach with initial complete surgical resection in limited disease. The prognosis is extremely poor.