Huge renal angiomyolipomas in tuberous sclerosis complex

Nephrology (Carlton). 2005 Aug;10(4):382-6. doi: 10.1111/j.1440-1797.2005.00425.x.

Abstract

Tuberous sclerosis complex (TSC) is an autosomal dominance disorder with variable penetrance. Renal angiomyolipoma (AML) is one of the commonest urological manifestations. These lesions may cause significant morbidity and mortality. We report two patients with multiple and huge renal angiomyolipomas presenting with gross haematuria. They subsequently underwent unilateral nephrectomy. The difficulty in diagnosis and controversies in management will be briefly discussed.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Angiomyolipoma / etiology*
  • Angiomyolipoma / pathology
  • Angiomyolipoma / therapy
  • Antigens, Neoplasm
  • Child
  • Female
  • Humans
  • Kidney Neoplasms / etiology*
  • Kidney Neoplasms / pathology
  • Kidney Neoplasms / therapy
  • Melanoma-Specific Antigens
  • Neoplasm Proteins / analysis
  • Tuberous Sclerosis / complications*
  • Tuberous Sclerosis Complex 1 Protein
  • Tuberous Sclerosis Complex 2 Protein
  • Tumor Suppressor Proteins / genetics

Substances

  • Antigens, Neoplasm
  • Melanoma-Specific Antigens
  • Neoplasm Proteins
  • Tuberous Sclerosis Complex 1 Protein
  • Tuberous Sclerosis Complex 2 Protein
  • Tumor Suppressor Proteins