title: Improvement of survival in patients with primary cutaneous diffuse large B-cell lymphoma, leg type, in France.
abstract: OBJECTIVE
  Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), occurs in elderly patients and has been considered as a lymphoma with a poor prognosis, with estimated 5-year specific survival rates of approximately 50%. The hypothesis of an improvement in prognosis over time has not been studied.
  
  
  OBJECTIVE
  To evaluate this hypothesis in a large series of patients and investigate factors associated with prognosis as well as improvement in the prognosis.
  
  
  METHODS
  A retrospective multicenter study was conducted including dermatology departments belonging to the French Study Group on Cutaneous Lymphoma. Participants were 115 patients with PCDLBCL-LT diagnosed between 1988 and 2003 (period 1) or between 2004 and 2010 (period 2).
  
  
  METHODS
  Age, sex, period of diagnosis, number of skin lesions, tumor stage, tumor location (leg vs nonleg), lactate dehydrogenase level, type of therapy (with or without a combination of rituximab and polychemotherapy [PCT]), and outcome were recorded. Baseline characteristics and outcome were compared according to period of diagnosis and type of therapy. Prognosis factors were identified by univariate and multivariate survival analyses.
  
  
  RESULTS
  The mean age of the patients was 76.9 years, and 47% of the patients were older than 80 years. The 3- and 5-year specific survival rates improved between period 1 and period 2, from 55% to 74% and from 46% to 66%, respectively (P = .01). Patients had similar baseline characteristics during both periods, but rituximab-PCT regimens were administered to 88.5% of the patients in period 2 vs 16.7% in period 1 (P < .001). The 3- and 5-year specific survival rates were 80% and 74%, respectively, in patients who received a rituximab-PCT regimen compared with 48% and 38% in those who received less-intensive therapies. No significant difference was observed between both groups in age and baseline prognostic factors. In multivariate analysis, treatment without rituximab-PCT was the only adverse prognostic factor (odds ratio, 4.6 [95% CI, 2.4-9.1]; P < .001), whereas the number of skin lesions (P = .06) and location on the leg (P = .07) had only borderline significance.
  
  
  CONCLUSIONS
  A major improvement in the survival of patients with PCDLBCL-LT has occurred over time in France, mainly as a result of the use of intensive rituximab-PCT regimens in most patients, including very elderly ones. Until further prospective clinical trials are conducted, such regimens should be considered as the standard of care in these patients.
authors.forename: Florent
authors.initials: F
authors.lastname: Grange
authors.forename: Pascal
authors.initials: P
authors.lastname: Joly
authors.forename: Coralie
authors.initials: C
authors.lastname: Barbe
authors.forename: Martine
authors.initials: M
authors.lastname: Bagot
authors.forename: Stéphane
authors.initials: S
authors.lastname: Dalle
authors.forename: Saskia
authors.initials: S
authors.lastname: Ingen-Housz-Oro
authors.forename: Eve
authors.initials: E
authors.lastname: Maubec
authors.forename: Michel
authors.initials: M
authors.lastname: D'Incan
authors.forename: Caroline
authors.initials: C
authors.lastname: Ram-Wolff
authors.forename: Sophie
authors.initials: S
authors.lastname: Dalac
authors.forename: Isabelle
authors.initials: I
authors.lastname: Templier
authors.forename: Eric
authors.initials: E
authors.lastname: Esteve
authors.forename: Gaëlle
authors.initials: G
authors.lastname: Quereux
authors.forename: Laurent
authors.initials: L
authors.lastname: Machet
authors.forename: Marion
authors.initials: M
authors.lastname: Leduc
authors.forename: Olivier
authors.initials: O
authors.lastname: Dereure
authors.forename: Liliane
authors.initials: L
authors.lastname: Laroche
authors.forename: Philippe
authors.initials: P
authors.lastname: Saiag
authors.forename: Béatrice
authors.initials: B
authors.lastname: Vergier
authors.forename: Marie
authors.initials: M
authors.lastname: Beylot-Barry
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000704:Analysis of Variance; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D002423:Cause of Death; D015331:Cohort Studies; D016001:Confidence Intervals; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D035002:Lower Extremity; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy* / Q000401:mortality* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D012189:Retrospective Studies; D018570:Risk Assessment; D012737:Sex Factors; D012878:Skin Neoplasms / Q000188:drug therapy* / Q000401:mortality* / Q000473:pathology; D016019:Survival Analysis; D013997:Time Factors