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

Abstract

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.

Clinical Differences between Benign and Malignant Pheochromocytomas.

“…In addition, malignant pheochromocytomas were nearly twice as large as benign tumours, with the former arising extraadrenally with greater frequency than the latter. Both observations confirm previous reports [9,12,13]. Twenty-four percent of the extraadrenal tumours in our study were malignant, versus only 8% of the intraadrenal tumours.…”

“…The malignancy rate of 6 to 20% reported by most authors [4][5][6][7][8][9][10][11][12][13][14] varies according to the definition of malignancy applied, i.e., metastasis, invasion of neighbouring organs, or invasion of organ capsules. Pheochro- Muenster, Germany mocytomas occur in less than 1 % of hypertensive individuals [15].…”

“…Female patients show a higher incidence of pheochromocytomas overall [4, 5, 8, 10-12, 14, 17, 22-24], but malignant pheochromocytomas occur more frequently in males [25], the latter being extraadrenal in the majority of cases [12][13][14].…”

Predictive Characteristics of Malignant Pheochromocytoma

“…We found that the incidence of malignant pheochromocytomas among 152 patients with pheochromocytomas was 11.2%, in good agreement with the 8% to 12.5% previously reported [7][8][9][10][11]. We also found that the 5-year survival rate of our patients with malignant pheochromocytoma was 11.8%, which was thus somewhat lower than the 20% previously reported [12].…”

Malignant Pheochromocytoma Lacking Clinical Features of Catecholamine Excess Until the Late Stage.

“…The reported incidence varies from less than 1% to 19% of all pheochromocytomas (1)(2)(3)(4)(5), and when the primary lesion is extraadrenal, this may rise to 24% (6) or 30% (7). Recurrence usually occurs several years after removal of the primary tumor (5,8,9), and bone is the most commonsite for metastases (8).…”