Diagnosis and management of asymptomatic renal cell carcinomas in von Hippel-Lindau syndrome

Urology. 1983 Feb;21(2):146-50. doi: 10.1016/0090-4295(83)90010-9.

Abstract

Renal cell carcinomas occurred in 6 of 17 subjects with the von Hippel-Lindau syndrome (HLS). In 4 patients, the tumors were discovered by abdominal computed tomography (CT) during a prospective study. Abdominal CT is recommended as a safe and accurate method for screening patients with HLS for renal cell carcinoma. Renal cell carcinoma in HLS tends to be multifocal and bilateral. Because of this, it is recommended that the tumors be treated by surgical enucleation rather than by more extensive surgical procedures which might ultimately lead to the need for dialysis.

MeSH terms

  • Adenocarcinoma / diagnosis*
  • Adenocarcinoma / genetics
  • Adenocarcinoma / surgery
  • Adult
  • Angiomatosis / complications*
  • Cysts / diagnostic imaging
  • Cysts / pathology
  • Female
  • Humans
  • Kidney / diagnostic imaging
  • Kidney / pathology
  • Kidney Diseases / diagnostic imaging
  • Kidney Diseases / pathology
  • Kidney Neoplasms / diagnosis*
  • Kidney Neoplasms / genetics
  • Kidney Neoplasms / surgery
  • Male
  • Middle Aged
  • Neoplasms, Multiple Primary / diagnosis*
  • Neoplasms, Multiple Primary / diagnostic imaging
  • Neoplasms, Multiple Primary / surgery
  • Prospective Studies
  • Tomography, X-Ray Computed
  • von Hippel-Lindau Disease / complications*
  • von Hippel-Lindau Disease / genetics