Can pheochromocytoma be considered a benign unilateral intra-adrenal sporadic hypertensive tumor ?
In a 20 year period, from 1971 through 1991, 105 chromaffin tumours–excluding cervical ones–were operated by the same surgeon: 50 during the first 15 years and 55 during the last 5 years. Pheochromocytomas are defined as intra-adrenal chromaffin tumours, and paragangliomas–or ectopic pheochromocytomas–as of extra-adrenal location. Among those tumours, 30 were malignant (i.e. metastatic) and 75 benign. Among the 30 malignant tumours, 14 were ectopic, 2 occurred in a MEN II A setting and were bilateral, 2 were associated with liver adenoma and liver hemangioma respectively suggesting Von Hippel-Lindau syndrome, and one case was associated with a seemingly sporadic primary hyperparathyroidism. 9 out of those 30 malignancies were not associated with hypertension. Among 75 benign pheochromocytomas or paragangliomas, 10 were ectopic, 7 occurred in a MEN setting (6 type II, 1 type I). 3 patients without evidence of MEN or other neuroectodermal abnormalities presented bilateral pheochromoc