October 1982

Intrathoracic and Multiple Abdominal Pheochromocytomas in Von Hippel–Lindau Disease

Author Affiliations

From the Departments of Medicine (Drs Hoffman and Gardner) and Surgery (Dr Mitchell) and the Division of Endocrinology (Dr Gardner), the University of Missouri School of Medicine, Columbia.

Arch Intern Med. 1982;142(10):1962-1964. doi:10.1001/archinte.1982.00340230212038

• A patient had von Hippeln—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.

(Arch Intern Med 1982;142:1962-1964)