[Renal angiomyolipoma and pulmonary lymphangiomyomatosis: a non-fortuitous association]

Rev Med Interne. 1994 Apr;15(4):279-81. doi: 10.1016/s0248-8663(94)80033-2.
[Article in French]

Abstract

Angiomyolipoma is a rare tumor, frequently associated with tuberous sclerosis, but not observed in its isolated form. Usually, patients with tuberous sclerosis presented with pathognomonic cutaneous and central nervous system lesions. But the thesis of "formes frustes" without typical stigmata was suggested. A case of bilateral and multifocal angiomyolipoma in a young woman without evidence of phacomatosis is reported. The association with a pulmonary lymphangiomatosis make us suspected "a forme fruste" of tuberous sclerosis.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adult
  • Angiomyolipoma / etiology
  • Angiomyolipoma / pathology*
  • Female
  • Humans
  • Kidney Neoplasms / etiology
  • Kidney Neoplasms / pathology*
  • Lung Neoplasms / etiology
  • Lung Neoplasms / pathology*
  • Lymphangioleiomyomatosis / etiology
  • Lymphangioleiomyomatosis / pathology*
  • Neoplasms, Multiple Primary*
  • Tuberous Sclerosis / complications
  • Tuberous Sclerosis / diagnosis*