Radiologic and clinical evaluation of pheochromocytoma in children: report of 6 cases.

Abstract

Pheochromocytomas arise from the chromaffin tissue of the adrenal medulla and paraspinal sympathetic ganglia. These tumors in rare instances occur in the brain (1) and neck (2) and, in a few cases, the bladder (3). Fewer than 5 per cent are found in the pediatric age group (4), and little has appeared in the radiologic literature about their manifestations in childhood. Approximately 100 cases of pheochromocytoma were reported in children by 1963 (5–7), the first case by Marchetti in 1904 (8). This paper describes 6 new cases in children and reviews briefly the clinical and roentgen features of childhood pheochromocytoma. Case Reports Case I: A n-year-old girl was admitted for evaluation of enuresis and high blood pressure of six months duration. Nose bleeds and headaches had been frequent since the age of five, and she had lost weight in recent months. Her father suffered from hypertension. Physical examination revealed a Grade III harsh systolic murmur to the left of the sternal border, as well as slight retinal arteriolar spasm. Her blood pressure was 140/110 with no difference between the arms and the legs. Fasting blood sugar was, on separate occasions, 120 mg per 100 cc and 94 mg per 100 cc, and the glucose tolerance test was abnormal. Urinary VMA (vanillyl mandelic acid) was elevated. Intravenous urography showed the right kidney to be slightly lower and 3 em shorter than the left, without a difference in rate of excretion of contrast material (Fig. 1). Aortography, using the Seldinger technic, demonstrated a mass in the right adrenal gland (Fig. 2). In the arterial phase, hypertrophied inferior suprarenal arteries supplied the pheochromocytoma. There was slight superior indentation of the distal half of the right renal artery, which was decreased in caliber by the adjacent tumor. In the late arterial phase, multiple irregularly arranged tumor vessels were seen. Still later an intense tumor blush outlined the lesion. During the induction of anesthesia for surgery, a hypertensive crisis and then shock developed. Blood transfusions were given, and pulmonary edema ensued. The patient recovered from these complications and was subjected to exploratory surgery ten days later after preoperative preparation with oral phentolamine (Regitine); a 3-cm pheochromocytoma was removed from the right suprarenal gland. The child's blood pressure fell postoperatively to 120/80, and her VMA excretion also became normal. Case II: This 9-year-old boy came to the Children's Clinic for repair of an inguinal hernia and an undescended testicle. He had been listless and tired for a few years, and his appetite was poor. He also complained of episodes of mild-to-moderate chest pain over the precordium, left anterior chest, and left scapula. These pains often occurred after vigorous activity. He was pale and seemed to have a rapid heart beat during these episodes.