title: Platinum plus bortezomib for the treatment of pediatric renal medullary carcinoma: Two cases.
abstract: Renal medullary carcinoma (RMC) was first described over two decades ago as the seventh sickle nephropathy. Survival after diagnosis with metastatic disease still rarely extends beyond 1 year, with recent reports of median overall survival in patients treated with platinum therapy being just 10 months. We describe our experience using platinum-based chemotherapy plus the proteasome inhibitor bortezomib to treat metastatic RMC in two pediatric patients who had complete responses. One patient passed away 7 years after diagnosis, while another remains disease free nearly 2 years from diagnosis.
authors.affiliation: Department of Pediatric Hematology/Oncology, Emory University School of Medicine, Children's Healthcare of Atlanta, Aflac Cancer & Blood Disorders Center, Atlanta, Georgia.
authors.forename: Marcus A
authors.initials: MA
authors.lastname: Carden
authors.affiliation: Department of Pediatric Hematology/Oncology, University of Kansas Medical Center.
authors.forename: Stephen
authors.initials: S
authors.lastname: Smith
authors.affiliation: Department of Hematology/Oncology, Children's National Medical Center.
authors.forename: Holly
authors.initials: H
authors.lastname: Meany
authors.affiliation: Department of Pathology, Children's Healthcare of Atlanta.
authors.forename: Hong
authors.initials: H
authors.lastname: Yin
authors.affiliation: Department of Radiology and Imaging Sciences, Children's Healthcare of Atlanta and Emory University School of Medicine.
authors.forename: Adina
authors.initials: A
authors.lastname: Alazraki
authors.affiliation: Department of Pediatric Hematology/Oncology, Emory University School of Medicine, Children's Healthcare of Atlanta, Aflac Cancer & Blood Disorders Center, Atlanta, Georgia.
authors.forename: Louis B
authors.initials: LB
authors.lastname: Rapkin
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D000069286:Bortezomib / Q000008:administration & dosage; D018276:Carcinoma, Medullary / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D002648:Child; D002945:Cisplatin / Q000008:administration & dosage; D003841:Deoxycytidine / Q000008:administration & dosage / Q000031:analogs & derivatives; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality; D008297:Male; D017239:Paclitaxel / Q000008:administration & dosage; D000093542:Gemcitabine
chemical_list: D003841:Deoxycytidine; D000069286:Bortezomib; D017239:Paclitaxel; D002945:Cisplatin; D000093542:Gemcitabine
keywords: bortezomib; platinum chemotherapy; renal medullary carcinoma; sickle cell trait