Rare forms of pheochromocytoma in children

Two cases of pheochromocytomas in multiple locations are described. In the first case, a 5-year-old girl had surgery for a pheochromocytoma that was nonsecreting but had obstructed the renal artery. Ten years later, hypertension recurred and 2 adrenal pheochromocytomas, one on the left and one on the right side, were discovered. In addition, a papillary angioma was present in the fundus of the right eye, which indicated a phacomatosis. The child was cured. In the second case, another 5-year-old girl had a secreting pheochromocytoma in the upper thoracic region. Six months after extirpation of the tumour, a second pheochromocytoma was found in the right adrenal gland. These findings indicate 4 points of interest: 1. The thoracic localization. This prompted a review of all the available literature with the conclusion that this form of tumour is extremely rare. 2. The association of a pheochromocytoma and renal arterial stenosis. This has been observed by a number of other authors as well. 3. The coincidence of a pheochromocytoma as part of a phacomatosis and pheochromocytomas associated with Sipple's syndrome. 4. The multiple tumour sites. The necessity of generalized examinations in patients with pheochromocytomas to determine the presence of a phacomatosis or Sipple's syndrome is apparent.