Most patients develop a pheochromocytoma from unknown causes. Approximately 10% to 20% of pheochromocytomas may be associated with an inherited autosomal dominant trait, which resulted in two familial multiple endocrine neoplasia (MEN) syndromes. Type Ila MEN combines pheochromocytoma with hyperparathyroidism and medullary carcinoma of the thyroid. Type IIb MEN combines pheochromocytoma and medullary carcinoma of the thyroid with multiple neuromas, Marfan's syndrome, hypertrophic corneal nerves, and ganglioneuromas.

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