title: Management and outcomes of patients with renal medullary carcinoma: a multicentre collaborative study.
abstract: To describe the management strategies and outcomes of patients with renal medullary carcinoma (RMC) and characterise predictors of overall survival (OS).
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Amishi Y
authors.initials: AY
authors.lastname: Shah
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Jose A
authors.initials: JA
authors.lastname: Karam
authors.affiliation: Groupe Hospitalier Pitié-Salpêtrière, University Pierre and Marie Curie, Paris, France.
authors.forename: Gabriel G
authors.initials: GG
authors.lastname: Malouf
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Priya
authors.initials: P
authors.lastname: Rao
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Zita D
authors.initials: ZD
authors.lastname: Lim
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Eric
authors.initials: E
authors.lastname: Jonasch
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Lianchun
authors.initials: L
authors.lastname: Xiao
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Jianjun
authors.initials: J
authors.lastname: Gao
authors.affiliation: Karmanos Cancer Center, Detroit, MI, USA.
authors.forename: Ulka N
authors.initials: UN
authors.lastname: Vaishampayan
authors.affiliation: Tom Baker Cancer Center, Calgary, Canada.
authors.forename: Daniel Y
authors.initials: DY
authors.lastname: Heng
authors.affiliation: Fox Chase Cancer Center, Philadelphia, PA, USA.
authors.forename: Elizabeth R
authors.initials: ER
authors.lastname: Plimack
authors.affiliation: University of Rochester, Rochester, NY, USA.
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Guancial
authors.affiliation: University of Rochester, Rochester, NY, USA.
authors.forename: Chunkit
authors.initials: C
authors.lastname: Fung
authors.affiliation: University of Nebraska Medical Center and Children's Hospital and Medical Center, Omaha, NE, USA.
authors.forename: Stefanie R
authors.initials: SR
authors.lastname: Lowas
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Pheroze
authors.initials: P
authors.lastname: Tamboli
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Kanishka
authors.initials: K
authors.lastname: Sircar
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Surena F
authors.initials: SF
authors.lastname: Matin
authors.affiliation: Vanderbilt-Ingram Cancer Center, Nashville, TN, USA.
authors.forename: W
authors.initials: W
authors.lastname: Kimryn Rathmell
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Christopher G
authors.initials: CG
authors.lastname: Wood
authors.affiliation: MD Anderson Cancer Center, Houston, TX, USA.
authors.forename: Nizar M
authors.initials: NM
authors.lastname: Tannir
mesh_terms: D000293:Adolescent; D000328:Adult; D018276:Carcinoma, Medullary / Q000188:drug therapy / Q000453:epidemiology / Q000401:mortality / Q000601:surgery; D002648:Child; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D007680:Kidney Neoplasms / Q000188:drug therapy / Q000453:epidemiology / Q000401:mortality / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009392:Nephrectomy; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: #KCSM; #KidneyCancer; kidney cancer; nephrectomy; renal cell carcinoma; renal medullary carcinoma (RMC)