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Pheochromocytomas are tumours of adrenal gland or extra-adrenal chromaffin tissues discovered in 0.1% of patients with hypertension.1 About 4% patients with incidentally discovered adrenal nodules are found to have pheochromocytoma.3 An overwhelming majority of these tumours are benign with malignancy reported in approximately 10% of cases at diagnosis.
The diagnosis of malignant PPGL requires finding this tumor in sites that do not normally contain chromaffin tissues (e.g., liver, bone, lung, omemtum, or lymph nodes).
Expression of phenylethanolamine N-methyltranspherase in rat sympathetic ganglia and extra-adrenal chromaffin tissue. Dev.
The area known as the organ of Zuckerkandl (aorticosympathetic group) encompasses all extra-adrenal chromaffin tissue near the origin of the inferior mesenteric artery and the aortic bifurcation.