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

Luigi Camera, Bernadette Biondi, Santa Daniele, Leonardo Pace, Loredana Di Nuzzo, Fabio Tortora, Melania Pulcrano, Loredana Pagano, Francesco Briganti, Gaetano Lombardi, Marco Salvatore

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

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.

Original languageEnglish
Title of host publicationEuropean Journal of Radiology Extra
Pages8-13
Number of pages6
Volume48
Edition1
DOIs
Publication statusPublished - Oct 2003

Keywords

  • Computed tomography
  • Helical
  • Paraganglioma
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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    Camera, L., Biondi, B., Daniele, S., Pace, L., Di Nuzzo, L., Tortora, F., Pulcrano, M., Pagano, L., Briganti, F., Lombardi, G., & Salvatore, M. (2003). Screening in von Hippel-Lindau disease: Concurrent pheochromocytomas, paragangliomas and spinal hemangioblastomas revealed by helical-CT, MIBG scintigraphy and MRI in an asymptomatic patient. In European Journal of Radiology Extra (1 ed., Vol. 48, pp. 8-13) https://doi.org/10.1016/S1571-4675(03)00079-8