[Pheochromocytoma and sympathetic paragangliomas]

Between 1971 and 1982, pheochromocytomas and sympathetic paragangliomas from 48 patients were examined at the Institute of Pathology of the University of Zurich. Thirty-four specimens had been resected surgically while 14 were discovered at autopsy, i.e. in 0.072% of the 19610 autopsies performed during that period. There were 42 cases (87.5%) of pheochromocytoma, of which 14.6% were bilateral, and 6 cases (12.5%) of sympathetic paragangliomas. The average age of our patients was 37.8% years, but the first clinical signs of the sympathetic paragangliomas preceded those of the pheochromocytomas by 20 years. Of all chromaffin tumors, 12.5% were obviously malignant and were metastasizing. In the assessment of biologic behavior it is noted that a histologically malignant tumor need not follow a malignant course and that, conversely, a tumor of benign appearance may develop metastases. Hence, long-term clinical follow-up is indicated as well as careful histologic diagnosis. The second part of this study will deal with associated diseases such as Sipple's disease and various neurocutaneous syndromes, which were present in 20.9% of our cases, and also with familial chromaffin tumors without associated diseases.