Androgen producing oncocytic adrenocortical tumor in a 3.7-year-old female.

Summary: We report a 3.7-year-old female who presented with clinical features of premature adrenarche. Workup revealed significantly elevated androgen levels and advanced bone maturation, prompting abdominal imaging that identified a 6.0 cm adrenal mass. She underwent unilateral adrenalectomy, and subsequent histopathology revealed a benign oncocytic adrenocortical adenoma. Post-adrenalectomy, androgen levels returned to pre-pubertal levels. A review of the literature on pediatric oncocytic adrenocortical neoplasms (OANs) is presented, and we add our case to the growing data of this rare disease in the pediatric population. Learning points: A high index of suspicion is necessary when encountering a prepubertal child with clinical signs of hyperandrogenism and advanced bone age. Oncocytic adrenocortical neoplasms are rare in children and are often benign, although they can be malignant or of uncertain malignant potential. Adrenalectomy is necessary for histopathological diagnosis and definitive treatment. Pediatric oncocytic adrenocortical neoplasms are typically associated with elevated androgens; however, they can have a variety of other abnormal hormonal profiles at presentation. Return to normal hormonal levels is common post-adrenalectomy.