Nonfunctional Adrenocortical Carcinoma with Foci of Osseous Metaplasia in a Young Girl

Nonfunctional adrenocortical carcinoma is an extremely rare malignant tumor in children. Unlike the functional tumor which is detected early due to its hormonal presentation, nonfunctional tumor is detected at a later stage. Here we report a case of a 10 year old girl who presented with abdominal mass and symptoms of short duration. No hypertension and cushingoid features were seen. Serum alpha-fetoprotein, urine vanillyl mandelic acid and homovanillic acid levels were not elevated. CT scan showed multiple pulmonary nodules suggestive of metastatic deposits. With gross and light microscopic findings differential diagnoses of hepatoblastoma, paraganglioma, renal cell carcinoma, adrenal cortical and medullary tumours were made. An array of immunohistochemical markers was done and the final diagnosis given was nonfunctional adrenocortical carcinoma with foci of osseous metaplasia.