Extra-adrenal intrathoracic functioning paraganglioma (pheochromocytoma) in childhood

Extra-adrenal pheochromocytomas are more common in children (30%) than in adults (10%). Of the extra-adrenal sites, the intrathoracic site is the most rare. A 15-year-old boy had a pheochromocytoma successfully removed from the left paraspinal region of his chest. Four years before removal, he had radiation therapy of 3,500 rads to the tumor because of its apparent nonresectability. After therapy, there was transient clinical and biochemical improvement. Preoperative angiography and computed tomography helped define the anatomy of the tumor vessels and the relationship to the thoracic aorta of the tumor. These studies also aided in discounting any other site for the pheochromocytoma, either adrenal or extramedullary. A slow-growing pulmonary metastatic lesion was subsequently identified and successfully excised.