Cushing syndrome secondary to CRH-producing Wilms tumor in a 6 year old.

[cushing syndrome]

Abstract Cushing syndrome is caused by prolonged exposure to elevated serum cortisol . It is uncommon in children , and etiology includes pituitary adenoma , adrenal tumor , and exogenous glucocorticoid administration . Rarely , it is paraneoplastic in origin . We present a case of paraneoplastic Cushing syndrome due to Wilms tumor that secreted corticotropin-releasing hormone (CRH ). A 6 -year -old male presented with polyphagia and weight gain . He showed Cushingoid appearance , hypertension , and palpable left flank mass . Serum cortisol and adrenocorticotropic hormone (ACTH ) levels were elevated . Computed tomography showed a neoplasm originating from the left kidney . Pathologic diagnosis of Wilms tumor was made upon nephroureterectomy . Immunohistochemical staining was positive for CRH and negative for ACTH . All features of Cushing syndrome disappeared after surgery . This represents a rare case of Cushing syndrome secondary to Wilms tumor in which CRH production has been demonstrated .