Ectopic production of ACTH by Wilms' tumor

The authors present the 2nd documented case of Wilms' tumor associated with the "ectopic ACTH syndrome'. This is a 7 1/2-year-old girl who, on examination at the time of admission, had the classical cushingoid appearance. A large hard mass was palpable in the right side of the abdomen. Hormonal assays were consistent with Cushing's syndrome; the serum ACTH levels were extremely high. After surgical removal of the mass, we suspected a stage I Wilms' tumor; this was confirmed by histopathological studies. After surgery, the girl quickly lost her cushingoid appearance and weight excess. Postoperative serum ACTH levels were normal. Ectopic hormone syndromes associated with tumors in childhood are discussed as well as the possible mechanism involved in the ectopic production of ACTH.