Current Urology Reports

, Volume 2, Issue 1, pp 24–30

Early identification of patients with von Hippel-Lindau disease at risk for pheochromocytoma

  • Jodi K. Maranchie
  • McClellan M. Walther
Article

DOI: 10.1007/s11934-001-0022-z

Cite this article as:
Maranchie, J.K. & Walther, M.M. Curr Urol Rep (2001) 2: 24. doi:10.1007/s11934-001-0022-z

Abstract

von Hippel-Lindau disease (VHL) is an autosomal dominant familial syndrome that predisposes to the formation of tumors in multiple organ systems, including adrenal and extra-adrenal pheochromocytomas. However, fewer than 30% of VHL families develop pheochromocytomas. In recent years, this clinical heterogeneity has been correlated with missense mutations. The VHL patient requires vigilant, lifelong biochemical and radiographic screening for pheochromocytoma. Half of VHL pheochromocytomas present bilaterally, and there is a high incidence of recurrence after surgery. Because of the morbidity of bilateral total adrenalectomy with subsequent steroid replacement therapy, the recent therapeutic trend has been toward observation and minimally invasive adrenal-sparing procedures.

Copyright information

© Current Science Inc 2001

Authors and Affiliations

  • Jodi K. Maranchie
    • 1
  • McClellan M. Walther
    • 1
  1. 1.Urologic Oncology BranchNational Cancer InstituteNorth BethesdaUSA