Cushing's syndrome secondary to ACTH-secreting Wilms'Tumor

In a review of the literature we only found one case of Wilms'tumor associated with “ectopic ACTH syndrome”. We are reporting the second documented case. A girl, 7 1/2 years old, who 5 months ago presented a sudden gain in apetite and corporal weight. On admission she had the classical cushingoid appearance and a blood pressure of 160/120 mmHg. A large hard mass was palpable in the right side of the abdomen. The urography and renal arteriography demonstrated the presence of a right renal mass. Hormonal assays were consistent with Cushing'syndrome; the serum ACTH levels were extremely high. After surgical removal of the mass, we suspected it to be a stage I Wilms'tumor; this was confirmed through histopathological studies. Chemotherapy was initiated following the protocol of The National Wilms'Tumor Study Group. The girl quickly lost her cushingoid appearance and weight excess. Postoperative serum ACTH levels were normal. In order to explain the ectopic ACTH synthesis mechanism several theories are actually on discussion, such us loss of genetic depressor mechanisms, presence of abnormal DNA, and APUD system.