title: A case-control study of clinicopathologic features, prognosis, and therapeutic responses in patients with granulomatous mycosis fungoides.
abstract: BACKGROUND
  Granulomatous mycosis fungoides (GMF) is an uncommon variant of mycosis fungoides (MF).
  
  
  OBJECTIVE
  We sought to analyze the relative frequency, clinicopathologic characteristics, prognosis, and therapeutic responses of GMF.
  
  
  METHODS
  We conducted a retrospective case-control study of patients with GMF and age- and stage-matched patients with classic MF between 1981 and 2012.
  
  
  RESULTS
  A total of 27 patients with GMF were identified, representing 6.3% of all patients with MF at our center. Skin manifestations were similar to classic MF having an atypical lichenoid CD4(+) CD8(-) lymphocytic infiltrate with interstitial histiocytes and/or perivascular granulomas with giant cells. Fewer patients with GMF achieved a partial response or complete response with topical (57% vs 83%; P = .002) or ultraviolet light (62% vs 90%; P = .006) therapy. The 5- and 10-year progression-free survival rates were significantly lower in patients with GMF (59% and 33%) compared with patients with classic MF (84% and 56%; P = .02), but overall survival was similar between groups (86% and 72% vs 85% and 85%; P = .54).
  
  
  CONCLUSIONS
  The retrospective methodology may underestimate the frequency of GMF. The median follow-up time may be too short to detect possible differences in overall survival.
  
  
  CONCLUSIONS
  More frequent disease progression and poorer response to skin-directed therapies were observed in patients with GMF. Our findings may be helpful in selecting the most appropriate treatment for these patients.
authors.affiliation: Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, New York 10022, USA.
authors.forename: Janet Y
authors.initials: JY
authors.lastname: Li
authors.forename: Melissa P
authors.initials: MP
authors.lastname: Pulitzer
authors.forename: Patricia L
authors.initials: PL
authors.lastname: Myskowski
authors.forename: Stephen W
authors.initials: SW
authors.lastname: Dusza
authors.forename: Steven
authors.initials: S
authors.lastname: Horwitz
authors.forename: Alison
authors.initials: A
authors.lastname: Moskowitz
authors.forename: Christiane
authors.initials: C
authors.lastname: Querfeld
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016022:Case-Control Studies; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006099:Granuloma / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000150:complications / Q000473:pathology* / Q000628:therapy; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000473:pathology* / Q000628:therapy; D015996:Survival Rate; D016896:Treatment Outcome; D014467:Ultraviolet Therapy; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents
keywords: CR; EORTC; European Organization for Research and Treatment of Cancer; GMF; ISCL; International Society for Cutaneous Lymphomas; LCT; MF; OS; PFS; PR; T-cell receptor; T-helper; TCR; Th; complete response; cutaneous T-cell lymphoma; disease progression; granulomatous mycosis fungoides; histopathology; incidence; large cell transformation; mycosis fungoides; outcome; overall survival; partial response; progression-free survival; treatment response