PEDIATRICS Vol. 28 No. 4 October 1961, pp. 566-577
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CUSHING'S SYNDROME IN ASSOCIATION WITH RENAL GANGLIONEUROBLASTOMA

Maurice D. Kogut M.D.1 and George N. Donnell M.D.2

1 Childrens Hospital of Los Angeles
2 The Department of Pediatrics, University of Southern California

A 13-month-old female with ganglioneuroblastoma originating in the lower pole of the left kidney has been described. This patient had the classic findings of Cushing's syndrome, namely, moon facies, buffalo hump, hypertrichosis, acne, plethora, hypertension, polyuria, polydypsia, osteoporosis and an elevated blood sugar concentration. In addition, there was increased excretion of 17-ketogenic steroids, 17-ketosteroids, aldosterone and vanillylmandelic acid in urine. After removal of the tumor, the abnormal clinical findings and the abnormally high excretion rate of corticosteroids and vanillylamandelic acid returned to normal. The adrenal glands were not hyperplastic nor were they the site of tumor formation.

The possible role of catecholamines in the pathogenesis of Cushing's syndrome is discussed. A most likely explanation for the association in this case is that catecholamines secreted by the tumor stimulated the adrenals or pituitary causing hyperactivity and Cushing's syndrome. Several cases have been cited that imply a closer functional relationship between chromaffin tissue and adrenal cortex than has been previously realized. The embryology of neurogenic tissue and its relation to secretion of catecholamines is discussed.