Renal medullary carcinoma: molecular, pathological and clinical evidence for treatment with topoisomerase-inhibiting therapy
Version of Record online: 11 DEC 2009
© 2009 THE AUTHORS. JOURNAL COMPILATION © 2009 BJU INTERNATIONAL
Volume 106, Issue 1, pages 62–65, July 2010
How to Cite
Schaeffer, E. M., Guzzo, T. J., Furge, K. A., Netto, G., Westphal, M., Dykema, K., Yang, X., Zhou, M., Teh, B. T. and Pavlovich, C. P. (2010), Renal medullary carcinoma: molecular, pathological and clinical evidence for treatment with topoisomerase-inhibiting therapy. BJU International, 106: 62–65. doi: 10.1111/j.1464-410X.2009.09139.x
- Issue online: 7 JUN 2010
- Version of Record online: 11 DEC 2009
- Accepted for publication 9 September 2009
- Renal medullary carcinoma;
Study Type – Aetiology (case series) Level of Evidence 4
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.
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.
This report provides molecular evidence for the rational use of TopoII inhibitors in the treatment of RMC.