Phaeochromocytoma in children.

  title={Phaeochromocytoma in children.},
  author={Ruth K. Armstrong and Meera Sridhar and Katriona Greenhalgh and Lucy Howell and Christine Jones and Christian Landes and Jo L McPartland and Carolyn Moores and Paul D Losty and Mohammed Ali Didi},
  journal={Archives of disease in childhood},
  volume={93 10},
Phaeochromocytoma is a rare clinical entity in children. Contrary to traditional teaching, which suggested that 10% of phaeochromocytomas are "familial", a germline mutation has been identified in up to 59% (27/48) of apparently sporadic phaeochromocytomas presenting at 18 years or younger and in 70% of those presenting before 10 years of age. The inherited predisposition may be attributable to a germline mutation in the Von Hippel-Lindau gene, the genes encoding the subunits B and D of… CONTINUE READING


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