Renal medullary carcinoma: Molecular, pathological and clinical evidence for treatment with topoisomerase-inhibiting therapy

Academic Article


  • Study Type - Aetiology (case series) Level of Evidence4 OBJECTIVE To present the molecular rationale and potential clinical benefit of topoisomerase II (TopoII)-inhibiting therapy for renal medullary carcinoma (RMC), a rare but extremely lethal form of kidney cancer that classically afflicts young men with sickle-cell trait. The current therapeutic approach with these aggressive tumours is radical nephrectomy followed by systemic chemotherapy, but the prognosis remains dismal. MATERIALS AND METHODS The whole-genome expression was analysed in four RMC tumours. We also report a case of metastatic RMC in which a complete response was achieved for 9 months using a TopoII-inhibiting therapy. RESULTS Expanded whole-genome expression analysis showed increases of TopoII in all cases. There was also overall deregulation of DNA remodelling and repair, and an ontological association between RMC and urothelial carcinoma. Using a TopoII-inhibiting agent, there was a complete response for 9 months in a patient with metastatic RMC. CONCLUSION This report provides molecular evidence for the rational use of TopoII inhibitors in the treatment of RMC. © 2009 BJU INTERNATIONAL.
  • Published In

  • BJU International  Journal
  • Digital Object Identifier (doi)

    Author List

  • Schaeffer EM; Guzzo TJ; Furge KA; Netto G; Westphal M; Dykema K; Yang X; Zhou M; Teh BT; Pavlovich CP
  • Start Page

  • 62
  • End Page

  • 65
  • Volume

  • 106
  • Issue

  • 1