STUDY TYPE: Aetiology (case series) Level of Evidence 4. 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.
STUDY TYPE: Aetiology (case series) Level of Evidence 4. 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.
Authors: Simon Chowdhury; Marc R Matrana; Christopher Tsang; Bradley Atkinson; Toni K Choueiri; Nizar M Tannir Journal: Hematol Oncol Clin North Am Date: 2011-08 Impact factor: 3.722
Authors: Amishi Y Shah; Jose A Karam; Gabriel G Malouf; Priya Rao; Zita D Lim; Eric Jonasch; Lianchun Xiao; Jianjun Gao; Ulka N Vaishampayan; Daniel Y Heng; Elizabeth R Plimack; Elizabeth A Guancial; Chunkit Fung; Stefanie R Lowas; Pheroze Tamboli; Kanishka Sircar; Surena F Matin; W Kimryn Rathmell; Christopher G Wood; Nizar M Tannir Journal: BJU Int Date: 2016-12-09 Impact factor: 5.588
Authors: Pavlos Msaouel; Andrew L Hong; Elizabeth A Mullen; Michael B Atkins; Cheryl Lyn Walker; Chung-Han Lee; Marcus A Carden; Giannicola Genovese; W Marston Linehan; Priya Rao; Maria J Merino; Howard Grodman; Jeffrey S Dome; Conrad V Fernandez; James I Geller; Andrea B Apolo; Najat C Daw; H Courtney Hodges; Marva Moxey-Mims; Darmood Wei; Donald P Bottaro; Michael Staehler; Jose A Karam; W Kimryn Rathmell; Nizar M Tannir Journal: Clin Genitourin Cancer Date: 2018-09-12 Impact factor: 2.872