[Diagnosis and surgical treatment of extra-adrenal pheochromocytoma]

From 1980 until 1991 10 patients were operated upon a paraganglioma: 5 patients with an encapsulated solitary tumor, two with infiltrating tumors and 3 with metastatic disease. The diagnosis of a catecholamine-producing tumor was most reliably made by norepinephrine analysis in urine and serum. The localization of the tumor was diagnosed in 9 of 10 cases by sonography and CT-scan. In 9 patients the tumor was excised completely, 4 times by extended resections. Whereas 4 out of 5 patients with an encapsulated solitary paraganglioma live to date without recurrence, all 3 patients suffering from metastatic disease had recurrent disease within one year postoperatively. A differentiated operative therapy of paragangliomas based on a clinical characterization is proposed: Encapsulated paragangliomas should always be excised. For solitary infiltrating tumors an extended resection may be necessary. This seems not to be justified for multiple infiltrating or metastasizing paragangliomas. Thus, only a reduction in size may be adequate for these tumors.