Intrathoracic and multiple abdominal pheochromocytomas in von Hippel-Lindau disease

A patient had von Hippel-Lindau disease, a functional intrathoracic paraganglioma (pheochromocytoma), bilateral adrenal pheochromocytomas, and a para-adrenal pheochromocytoma. Seven other members of the patient's family had features of von Hippel-Lindau disease and one, a cousin, had medullary carcinoma of the thyroid. This is the first report of a pheochromocytoma arising outside the abdomen in von Hippel-Lindau disease and the 25th report of intrathoracic pheochromocytoma in the literature. The association between von Hippel-Lindau disease and pheochromocytoma is reviewed.