Isolation of Cortisol from a Pheochromocytoma ‡

Although the adrenal cortex and medulla arise from different embryological tissues, they are intimately associated in the developing embryo. The adrenal cortex arises from splanchnic mesenchyma, while the medulla arises from nervous tissue.' The chromaffin cells migrate by way of the celiac plexus to penetrate the adrenal cortex as scattered islands which later aggregate to form the adrenal medulla. It is conceivable that islands of chromaffin cells surrounded by adrenocortical cells might develop as aberrant tissue. Later, if a tumor developed in this aberrant tissue, both adrenocortical and medullary elements would be present. Clusters of cells which resemble cortical cells are occasionally seen in pheochromocytomas.7'9'10 Reports in the literature suggest that certain pheochromocytomas do secrete steroids. A striking case reported in 1942, by Neff and co-workers,"2 was that of a 16-month-old-girl with marked hirsutism, overdevelopment of external genitalia, obesity, and paroxysmal hypertension. Removal of an adrenal tumor from this patient resulted in an immediate fall in blood pressure and regression of all symptoms. Histologically, the tumor was a pheochromocytoma, with one peculiar microscopic feature: lipoid material within many cells. Chenualt, et al.6 described a patient with paroxysmal hypertension, diabetes mellitus, elevated urinary 17-hydroxycorticoid excretion, and an abnormally increased excretion of 17-hydroxycorticoids after an ACTH test. Symptoms and chemical findings returned to normal after removal of a tumor at the bifurcation of the aorta which contained 7.5 mg. of catecholamines per gm. of tumor with more than 6.2 mg. as norepinephrine.