Adrenocortical tumors (ACTs) are extremely rare in infants. Pediatric ACTs are therapeutic and diagnostic challenge because histological criteria for distinguishing benign from malignant tumors seen in adults are not always reliable in children .The aim of this report was to present clinical features, hormonal profile, and histopathological characteristics of an infant with Adrenal tumors.

A 10-month-old boy with adrenocortical tumors (ACT) presented with restlessness and pseudoprecocious puberty. Plasma testosterone, dehydroepiandrosterone, and 17-OH progesterone were increased. An abdominal spiral computed tomography (CT) scan with intravenous contrast identified a well-defined left-sided suprarenal mass with areas of necrosis. Left adrenalectomy was performed. Immunohistochemical staining showed that tumor cells were strongly positive with inhibin and focal positive with vimentin. The diagnosis of adrenocortical carcinoma was made on the basis of immune histochemistry. The postoperative course was uneventful.

This case emphasized the importance of early consideration of an adrenocortical tumors causing pseudoprecocious puberty in infant. The final pathologic diagnosis should be based on imunohistochemical staining patterns.