Metastatic Pheochromocytoma in an Asymptomatic 12-Year-Old With von Hippel-Lindau Disease.

Pheochromocytoma is a rare chromaffin cell tumor that may be associated with a genetic predisposition, such as von Hippel-Lindau (VHL) disease. VHL is an autosomal dominant disorder that is characterized by a predisposition to multiple tumors, including retinal and central nervous system hemangioblastomas, renal cell carcinoma, and pheochromocytomas. The classic presentation of pheochromocytoma is episodic hypertension, headaches, palpitations, and diaphoresis. In the pediatric population, 40% of pheochromocytomas have a hereditary basis. We present a case of metastatic pheochromocytoma in a child with VHL and discuss the relevant current medical literature.