What Is Extra-adrenal Pheochromocytomas?

Q:
What is extra-adrenal pheochromocytomas?

A:
Extra-adrenal pheochromocytomas are closely related, though less common, tumors that originate in the ganglia of the sympathetic nervous system Extra-adrenal pheochromocytomas usually weigh 20 to 40 g and are <5 cm in diameter. Most are located within the abdomen.

It accounts for nearly 10% of pheochromocytomas in adults and 30% in children. These are more likely to be malignant than adrenal pheochromocytomas (36% vs. 10%).

Pheochromocytoma is seen in between two and eight in 1,000,000, with approximately 1000 cases diagnosed in United States yearly. It mostly occurs in young or middle age adults, though it presents earlier in hereditary cases.

Among them, about 15% are extra-adrenal: Of these 9% are in the abdomen, and 1% are located elsewhere. Some extra-adrenal pheochromocytomas are probably actually paragangliomas, but the distinction can only be drawn after surgical resection.

Keyword: extra adrenal pheochromocytoma