Screening in von Hippel-Lindau disease: concurrent pheochromocytomas, paragangliomas and spinal hemangioblastomas revealed by helical-CT, MIBG scintigraphy and MRI in an asymptomatic patient

Early detection of visceral as well as neurological abnormalities in von Hippel-Lindau (VHL) disease is highly recommended and usually accomplished by a combination of radiological and nuclear medicine techniques. We report a case of an asymptomatic 30-year-old male with VHL disease who was found to have concurrent bilateral pheochromocytomas, a carotid body tumor, sacral para-gangliomas and spinal hemangioblastomas during a screening performed by contrast-enhanced helical computed tomography, 131I-metaiodobenzylguanidine scintigraphy and magnetic resonance imaging. Despite their common embriogenetic origin from the neural crest, the concurrence of pheochromocytomas and para-gangliomas in VHL disease is rare. Although frequently asymptomatic, the occurrence of spinal hemangioblastomas in the clinical context of the screening has not been previously reported.