SUMMARY: An infant with a Wilms tumor and severe hypertension was studied to evaluate the concept that a humoral substance secreted by the tumor caused the hypertension.
Abnormal glucose tolerance, hypokalemia, decreased insulin release, positive response to intravenously administered phentolamine, elevated plasma renin concentrations, normal urinary aldosterone, and increased vanillylmandelic acid levels were found. These abnormalities reverted towards normal postoperatively.
Assays of the tumor did not reveal the enzymes necessary for catecholamine biosynthesis, but it was inferred that a catecholamine-like substance plays a role in the hypertension and carbohydrate abnormalities sometimes associated with Wilms tumors and that this tumor may belong in the group of nonendocrine neoplasms that can directly or indirectly cause clinically significant endocrine activity.