One hundred cases of pheochromocytoma (107 tumors) at the columbia‐presbyterian medical center, 1926‐1976. A clinicopathological analysis

Among 100 patients with pheochromocytoma there were 89 adults (sexes about even in distribution) and 11 children (7 boys; 4 girls). The right adrenal was involved 54 times, about twice the incidence on the left (29 times), and in three patients the tumors were bilateral. Seventeen had extra‐adrenal pheochro‐mocytomas, of which nine arose in the organ of Zuckerkandl. Only two of the 83 intra‐adrenal pheochromocytomas, or 2.4%, were malignant; whereas five of the extra‐adrenal tumors, or 30%, were cancerous. Associated lesions such as neurofibromatosis, renal artery stenosis, and hibernating fat are discussed. The 100 patients were divided into two groups: 1) 83 with clinically functioning pheochromocytomas, and 2) 17 with clinically “silent” tumors. In the first there were 70 with functioning tumors correctly diagnosed, and 13, or one in six, in whom it was missed, usually with tragic consequences. Seven had exploratory surgery for some other presenting condition, such as pre‐eclampsia, renal artery stenosis, skin nodule, cystocele, fibroid uterus, breast lump, or bladder “tumor”. It has been estimated that in the United States there are about 18,000 individuals with functioning pheochromocytomas producing hypertension. If the number is correct, then about 2000‐3000 will go unrecognized. Such a course will be avoided when the vagaries in the clinical behavior of pheochromocytoma are generally appreciated, when the amenuensis is thorough, thus sharpening cognate clinical suspicion, and when catecholamine determinations are more frequently performed. The 17 silent tumors constitute a cryptic hard core: the majority were extra‐adrenal, larger than the intra‐adrenal tumors and more frequently malignant. Association of pheochromocytoma with or medullary carcinoma of the thyroid, either concurrent or separate, has been observed, particularly among kindred. Other tumors, such as those of the pituitary, adrenal cortex, Lindau's disease, cysts of the liver and pancreas, retinoblastoma, etc., have also been reported in association with pheochromocytoma. This widespread involvement of apparently unrelated organs by neoplasia, explains Pearse, 35 is probably the result of the migration of some toti‐potent neuroectodermal cells, which migrate with the endoderm and settle in the various derivatives. He labels these tumors Apudomas.