Two boys with four pheochromocytomas each.

Two teenage boys were treated at separate pediatric institutions for four separate pheochromocytomas over the last 15 years. The first operation in each boy was performed between 9 and 11 years of age, after they presented with severe hypertensive encephalopathy. One boy had separate adrenal tumors excised in 1968, and 3 and 11 years later, two more separate new left adrenal pheochromocytomas were again resected. They were not recurrent left adrenal tumors from residual pheochromocytoma-secreting tissue, because no visible tumor tissue was left behind at the completion of each previous operation, and he was clinically well for years between each tumor resection. The pathology was benign pheochromocytoma. He remains well since the last operation in 1979 but has a residual hemiplegia from the first tumor. The family history is negative. The second boy was first operated on in 1976 at age 11 years when two benign pheochromocytomas were removed from around the right renal artery and the left para-aortic area. He remained well for 6 years when he became hypertensive again (this time without encephalopathy), and had a right chest paravertebral pheochromocytoma removed and several weeks later a left adrenal tumor was also removed. He remains well and followed closely. His family history is negative.