title: Primary cutaneous diffuse large B-cell lymphoma, leg type: clinicopathologic features and prognostic analysis in 60 cases.
abstract: OBJECTIVE
  To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas.
  
  
  METHODS
  Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas.
  
  
  METHODS
  Nineteen departments of dermatology in 10 regions of France.
  
  
  METHODS
  Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas.
  
  
  METHODS
  Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Disease-specific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model.
  
  
  RESULTS
  Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable short-term outcome.
  
  
  CONCLUSIONS
  Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.
authors: Department of Dermatology, Hôpital Robert Debré, Avenue du Général Koenig, Reims 51100, France. fgrange@chu-reims.fr
authors: Florent
authors: F
authors: Grange
authors: Marie
authors: M
authors: Beylot-Barry
authors: Phillipe
authors: P
authors: Courville
authors: Eve
authors: E
authors: Maubec
authors: Martine
authors: M
authors: Bagot
authors: Béatrice
authors: B
authors: Vergier
authors: Pierre
authors: P
authors: Souteyrand
authors: Laurent
authors: L
authors: Machet
authors: Sophie
authors: S
authors: Dalac
authors: Eric
authors: E
authors: Esteve
authors: Isabelle
authors: I
authors: Templier
authors: Emmanuel
authors: E
authors: Delaporte
authors: Marie-Françoise
authors: MF
authors: Avril
authors: Caroline
authors: C
authors: Robert
authors: Stephane
authors: S
authors: Dalle
authors: Liliane
authors: L
authors: Laroche
authors: Michele
authors: M
authors: Delaunay
authors: Pascal
authors: P
authors: Joly
authors: Janine
authors: J
authors: Wechsler
authors: Tony
authors: T
authors: Petrella
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D007866:Leg; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012878:Skin Neoplasms / Q000175:diagnosis / Q000401:mortality / Q000473:pathology* / Q000628:therapy