title: Clinically nonmetastatic renal cell carcinoma with sarcomatoid dedifferentiation: Natural history and outcomes after surgical resection with curative intent.
abstract: OBJECTIVE
  Renal cell carcinoma with sarcomatoid dedifferentiation (sRCC) is an aggressive malignancy associated with a poor prognosis. Although existing literature focuses on patients presenting with metastatic disease, characteristics and outcomes for patients with localized disease are not well described. We aimed to evaluate postnephrectomy characteristics, outcomes, and predictors of survival in patients with sRCC who presented with clinically localized disease.
  
  
  METHODS
  An institutional review board-approved review from 1986 to 2011 identified 77 patients who presented with clinically localized disease, underwent nephrectomy, and had sRCC in their primary kidney tumor. Clinical and pathologic variables were captured for each patient. Overall survival (OS) and recurrence-free survival (RFS) were calculated for all patients and those who had no evidence of disease (NED) following nephrectomy, respectively. Comparisons were made with categorical groupings in proportional hazards regression models for univariable and multivariable analyses.
  
  
  RESULTS
  OS for the entire cohort (n = 77) at 2 years was 50%. A total of 56 (77%) patients of the 73 who has NED following nephrectomy experienced a recurrence, with a median time to recurrence of 26.2 months. On multivariable analysis, tumor stage, pathologically positive lymph nodes, and year of nephrectomy were significant predictors of both OS and recurrence-free survival. Limitations include the retrospective nature of this study and relatively small sample size.
  
  
  CONCLUSIONS
  Long-term survival for patients with sRCC, even in clinically localized disease, is poor. Aggressive surveillance of those who have NED following nephrectomy is essential, and further prospective studies evaluating the benefit of adjuvant systemic therapies in this cohort are warranted.
authors.affiliation: Department of Urology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Megan M
authors.initials: MM
authors.lastname: Merrill
authors.affiliation: Department of Urology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Christopher G
authors.initials: CG
authors.lastname: Wood
authors.affiliation: Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Nizar M
authors.initials: NM
authors.lastname: Tannir
authors.affiliation: Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Rebecca S
authors.initials: RS
authors.lastname: Slack
authors.affiliation: Department of Urology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Kara N
authors.initials: KN
authors.lastname: Babaian
authors.affiliation: Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Eric
authors.initials: E
authors.lastname: Jonasch
authors.affiliation: Department of Genitourinary Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Lance C
authors.initials: LC
authors.lastname: Pagliaro
authors.affiliation: Department of Urology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Zachary
authors.initials: Z
authors.lastname: Compton
authors.affiliation: Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Pheroze
authors.initials: P
authors.lastname: Tamboli
authors.affiliation: Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Kanishka
authors.initials: K
authors.lastname: Sircar
authors.affiliation: Department of Urology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Louis L
authors.initials: LL
authors.lastname: Pisters
authors.affiliation: Department of Urology, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Surena F
authors.initials: SF
authors.lastname: Matin
authors.affiliation: Department of Urology, The University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address: JAKaram@mdanderson.org.
authors.forename: Jose A
authors.initials: JA
authors.lastname: Karam
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002292:Carcinoma, Renal Cell / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D054337:Cell Dedifferentiation; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D007680:Kidney Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009392:Nephrectomy; D016896:Treatment Outcome
keywords: Nephrectomy; Renal cell carcinoma; Sarcomatoid