Extra-adrenal pheochromocytoma accounts for about 10% of all such tumors. These neoplasms usually produce norepinephrine exclusively, and the patient's symptoms are related to overproduction of norepinephrine. Our patient's symptoms are primarily related to the overproduction of epinephrine. Preoperatively, he was thought to have a left adrenal tumor. At operation, however, the tumor was found to arise from one of the left lumbar sympathetic ganglia. Chemical assay of the tumor confirmed the presence of epinephrine in the extra-adrenal site. We believe this is the first instance where chemical assay has proven the presence of epinephrine in an extra-adrenal location.
Mannix H, O'Grady WP, Gitlow SI. Extra-Adrenal Pheochromocytoma-Producing EpinephrineIts Physiologic Significance. Arch Surg. 1972;104(2):216-218. doi:10.1001/archsurg.1972.04180020096020