Pheochromocytoma in children--an update

Eight cases of pheochromocytoma in children were managed between 1958 and 1978. Headache, visual blurring, sweating, and hypertension were the most common findings. One patient presented with a hypertensive crisis during appendectomy. Three other children had a family history of pheochromocytoma and Hippel-Landau disease, thyroid carcinoma, and renal stones. The diagnostic approach was based on a high suspicion of the disease from the clinical picture. The most reliable laboratory investigations were urinary VMA, metanephrine, and catecholamines. I.V.P. and angiography were successful in localizing the tumor. In 4 cases, CT scan localized the tumor, although a second tumor was not seen in 1 case. Preoperative control of hypertension was achieved with phenoxybenzamine, propanolol, apresoline and reserpine in seven cases. The main anesthetic drugs used were pentothal, methoxyflurane, nitrous oxide, Innovar, and pancuronium. Tumors were located in the right adrenal in four, the left adrenal in two, both adrenals in one, and bilateral para-aortic sites in one. In two cases, hypertensive episodes during manipulation of the tumor were controlled with phentolamine. Ligation of the venous drainage from a tumor was associated with a sudden sustained fall in systemic blood pressure in six cases. In two, blood pressure remained elevated until a second tumor was found and removed. Hypotension was then corrected by the rapid infusion of fluid and blood, and vasopressors were not used. There were no postoperative complications and all children remained normotensive during the follow-up. The tumors of seven of the patients were benign. One tumor showed a low grade malignancy.