[Pheochromocytoma and sympathetic paraganglioma. 2. Combination with typical associated diseases. Familial occurrence].

Of 48 patients with chromaffin tumors observed at the Institute of Pathology of the University of Zurich between 1971 and 1982, 10 (20.9%) had a typical associated disease: 6 patients (12.5%) had Sipple's syndrome, 2 von Recklinghausen's neurofibromatosis, and 2 others von Hippel-Lindau's disease. The origin common to all these disorders involves the neuroectoderm. In the presence of one of these diseases the possibility of an occult chromaffin tumor must be considered. Familial occurrence of chromaffin tumors was observed in 5 patients (10.5%): 3 (of two families) had Sipple's syndrome, while the other 2 (of one family) had neoplasms without associated diseases.