Pheochromocytoma and paraganglioma syndromes: genetics and management update.

  title={Pheochromocytoma and paraganglioma syndromes: genetics and management update.},
  author={M. Lefebvre and William D Foulkes},
  journal={Current oncology},
  volume={21 1},
Pheochromocytomas (pheos) and paragangliomas (pgls) are rare tumours of the autonomic nervous system, originating from paraganglia, which are dispersed neuroendocrine organs characterized by catecholamine and peptide-producing cells derived from the neural crest. Medical textbooks have traditionally suggested that 10% of pheos are heritable. However, the frequency of heritable pheo has been underestimated. Three syndromic conditions-Von Hippel-Lindau (vhl), multiple endocrine neoplasia type 2… CONTINUE READING