From the Archives of the AFIP

Giant non-functioning adrenocortical carcinoma: A rare childhood tumor

“…Adrenal tumors in children can be associated with hemihypertrophy and Beckwith Wiedmann syndrome. [3]…”

“…The common metastatic sites include lung and liver, with bone and bone marrow being less common. [3] Neuroblastoma, which is a more common childhood tumor with similar location, has a tendency to metastasize to bone and bone marrow, though there are case reports showing increasing prevalence of lung metastasis. [8] However, in neuroblastoma, pulmonary metastasis is usually a terminal event where at least one other metastatic site apart from lung is present, which could be bone, bone marrow or liver.…”

“…It accounts for 0.002% of childhood malignancies, with most of the tumors being functional in children. [3] Nonfunctioning adrenocortical tumors are exteremly rare in children. [3] There are many case reports for functional ACC, but very few reports are available for non-functioning ACC in children owing to its rarity.…”

“…[3] Nonfunctioning adrenocortical tumors are exteremly rare in children. [3] There are many case reports for functional ACC, but very few reports are available for non-functioning ACC in children owing to its rarity. [45]…”

“…In most of the cases, it is mistaken for neuroblastoma which is the commonest intra-abdominal childhood tumor. [3] Very rare incidence and unusual mode of presentation in childhood in our patient with a single functioning kidney prompted us to make a case report with its review of literature.…”