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Learn about the characteristics, incidence, and potential complications of bilateral adrenal pheochromocytomas, a rare but potentially fatal tumor. Discover the Rule of 10's and the associations with multiple endocrine neoplasia (MEN2).
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Pheochromocytoma Rule of 10’s • 10% Found incidentally • 10% Familial (MEN2A, MEN2B) • 10% Extra-adrenal • 10% Bilateral (multiple) • 10% Calcify • 10% Children • 10% Recurrence • 10% Malignant
Multiple Endocrine Neoplasia2 (MEN2) • MEN2A • Medullary thyroid cancer • Pheochromocytoma • Parathyroid adenoma • MEN2B • Medullary thyroid cancer • Pheochromocytoma • Mucosal neuromas (lips and tongue) • Marfanoid habitus
Pheochromocytoma • Rare but fatal catecholamines producing tumor • Incidence: 2-8/million people/year • Account for 4% of adrenal incidentaloma • Rule of 10’s: • 10% bilateral, 10% extra-adrenal, 10% familial, 10% malignant • Aside from catecholamines, it can also secrete dopamine, ACTH, PTH, calcitonin, vaso-intestinal polypeptide
Pheochromocytoma • Rare but fatal catecholamines producing tumor • Incidence: 2-8/million people/year • Account for 4% of adrenal incidentaloma • Rule of 10’s: • 10% bilateral, 10% extra-adrenal, 10% familial, 10% malignant • Aside from catecholamines, it can also secrete dopamine, ACTH, PTH, calcitonin, vaso-intestinal polypeptide