Rule of ten in pheochromocytoma

What is the rule of ten in pheochromocytoma?

Classically, each of the following accounts for about 10% of all pheochromocytomas:

• Bilateral (in both adrenal glands)

• Extra-adrenal

• Malignant

• Familial (associated with multiple endocrine neoplasia [MEN] syndromes)

• Pediatric

In reality, few large series show the expected 10% incidence; recent data suggest that about 20% of pheochromocytomas are heredofamilial (see next question).


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