Malignant transformation of renal angiomyolipoma

Int J Urol. 2003 May;10(5):271-3. doi: 10.1046/j.1442-2042.2003.00620.x.


In the present paper, two cases of malignant transformation of renal angiomyolipoma without tuberous sclerosis are reported. Pathological examination revealed that, in both cases, in addition to the areas affected by typical angiomyolipoma, there were areas that contained elevated numbers of perivascular epithelioid cells with prominent nuclear pleomorphism. Immunohistochemical examination revealed that both cases were negative for keratin and epithelial membrane antigen, but were positive for the melanogenesis-related marker HMB-45. Metastatic diseases appeared 40 months after radical nephrectomy in the first case and 18 months in the second case.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Angiomyolipoma / pathology*
  • Angiomyolipoma / surgery
  • Fatal Outcome
  • Female
  • Humans
  • Kidney Neoplasms / pathology*
  • Kidney Neoplasms / surgery
  • Liver Neoplasms / secondary*
  • Lung Neoplasms / secondary*
  • Male
  • Nephrectomy
  • Retroperitoneal Neoplasms / secondary*
  • Retroperitoneal Neoplasms / surgery
  • Splenic Neoplasms / secondary*
  • Splenic Neoplasms / surgery