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Shah AY , Karam JA , Malouf GG , Rao P , Lim ZD , Jonasch E , Xiao L , Gao J , Vaishampayan UN , Heng DY , Plimack ER , Guancial EA , Fung C , Lowas SR , Tamboli P , Sircar K , Matin SF , Kimryn Rathmell W , Wood CG , Tannir NM
Management and outcomes of patients with renal medullary carcinoma: a multicentre collaborative study
BJU Int. 2017 Dec;120(6) :782-792
PMID: 27860149    PMCID: PMC6857178    URL: https://www.ncbi.nlm.nih.gov/pubmed/27860149
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OBJECTIVE: To describe the management strategies and outcomes of patients with renal medullary carcinoma (RMC) and characterise predictors of overall survival (OS). PATIENTS AND METHODS: RMC is a rare and aggressive malignancy that afflicts young patients with sickle cell trait; there are limited data on management to date. This is a study of patients with RMC who were treated in 2000-2015 at eight academic institutions in North America and France. The Kaplan-Meier method was used to estimate OS, measured from initial RMC diagnosis to date of death. Cox regression analysis was used to determine predictors of OS. RESULTS: In all, 52 patients (37 males) were identified. The median (range) age at diagnosis was 28 (9-48) years and 49 patients (94%) had stage III/IV. The median OS for all patients was 13.0 months and 38 patients (75%) had nephrectomy. Patients who underwent nephrectomy had superior OS compared to patients who were treated with systemic therapy only (median OS 16.4 vs 7.0 months, P < 0.001). In all, 45 patients received chemotherapy and 13 (29%) had an objective response; 28 patients received targeted therapies, with 8-week median therapy duration and no objective responses. Only seven patients (13%) survived for >24 months. CONCLUSIONS: RMC carries a poor prognosis. Chemotherapy provides palliation and remains the mainstay of therapy, but <20% of patients survive for >24 months, underscoring the need to develop more effective therapy for this rare tumour. In this study, nephrectomy was associated with improved OS.
1464-410x Shah, Amishi Y Karam, Jose A Malouf, Gabriel G Rao, Priya Lim, Zita D Jonasch, Eric Xiao, Lianchun Gao, Jianjun Vaishampayan, Ulka N Heng, Daniel Y Plimack, Elizabeth R Guancial, Elizabeth A Fung, Chunkit Lowas, Stefanie R Tamboli, Pheroze Sircar, Kanishka Matin, Surena F Kimryn Rathmell, W Wood, Christopher G Tannir, Nizar M Journal Article Multicenter Study P30 CA016672/CA/NCI NIH HHS/ England BJU Int. 2017 Dec;120(6):782-792. doi: 10.1111/bju.13705. Epub 2016 Dec 9.