Pediatric pheochromocytoma. A 36-year review

Fourteen children (10 boys and 4 girls, aged 8 to 17 years) had 20 pheochromocytomas treated over a 36-year period from 1959 to 1995 inclusive. Nine patients had 11 tumors before 1980; 5 children had 9 tumors up to 1987. There were no new children with pheochromocytomas at our hospital from 1988 to 1995. Hypertension, sweating, headache, and visual blurring were the most common symptoms and signs (average 5 months). The most reliable biochemical investigations were the urinary catecholamines and norepinephrine. Before 1980, intravenous pyelography and angiography were most successful in localizing the tumor, but since then ultrasonography and computerized tomography have been the radiological investigations of choice. Early involvement of the anesthesiologist in the preoperative control of the hypertension is essential; blood pressure (BP) control was achieved with phenoxybenzamine. The main anesthetic drugs used were: sodium thiopental, fentanyl, methoxyflurane, isoflurane, nitrous oxide, and metocurine. Sixteen tumors were adrenal and 4 were extra-adrenal (1 intrathoracic and 1 extradural). All except 2 tumors were completely resected; they ranged in size from 1.3 to 14 cm. Ligation of the tumor's venous drainage was usually associated with a sudden, temporary fall in systemic BP. There were 2 children with malignant tumors. Four patients had five recurrences (second pheochromocytoma) within 6 years, and all were heralded by a return of their original symptoms and signs. One girl was left with no adrenal tissue. The only complication was in a boy with a large, partly-resected malignant right adrenal tumor who had a subphrenic abscess drained and was left with a temporary bile fistula, cirrhosis, and chronic pain. All children were normotensive when discharged from hospital and remain alive and well with a follow-up of 7 to 36 years. There were no deaths. Long-term follow-up is essential. Key word Pheochromocytoma