title: Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC).
abstract: BACKGROUND
  Granulomatous cutaneous T-cell lymphomas (CTCLs) are rare and represent a diagnostic challenge. Only limited data on the clinicopathological and prognostic features of granulomatous CTCLs are available. We studied 19 patients with granulomatous CTCLs to further characterize the clinicopathological, therapeutic, and prognostic features.
  
  
  METHODS
  The group included 15 patients with granulomatous mycosis fungoides (GMF) and 4 with granulomatous slack skin (GSS) defined according to the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. Patients with GMF and GSS displayed overlapping histologic features and differed only clinically by the development of bulky skin folds in GSS. Histologically, epidermotropism of lymphocytes was not a prominent feature and was absent in 9 of 19 cases (47%). Stable or progressive disease was observed in most patients despite various treatment modalities. Extracutaneous spread occurred in 5 of 19 patients (26%), second lymphoid neoplasms developed in 4 of 19 patients (21%), and 6 of 19 patients (32%) died of their disease. Disease-specific 5-year survival rate in GMF was 66%.
  
  
  CONCLUSIONS
  There are clinical differences between GMF and GSS, but they show overlapping histologic findings and therefore cannot be discriminated by histologic examination alone. Development of hanging skin folds is restricted to the intertriginous body regions. Granulomatous CTCLs show a therapy-resistant, slowly progressive course. The prognosis of GMF appears worse than that of classic nongranulomatous mycosis fungoides.
authors.affiliation: Department of Dermatology, University Hospital Zürich, Gloriastrasse 31, CH-8091 Zürich, Switzerland. kempf@derm.unizh.ch.
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.forename: Sonja
authors.initials: S
authors.lastname: Ostheeren-Michaelis
authors.forename: Marco
authors.initials: M
authors.lastname: Paulli
authors.forename: Marco
authors.initials: M
authors.lastname: Lucioni
authors.forename: Janine
authors.initials: J
authors.lastname: Wechsler
authors.forename: Heike
authors.initials: H
authors.lastname: Audring
authors.forename: Chalid
authors.initials: C
authors.lastname: Assaf
authors.forename: Thomas
authors.initials: T
authors.lastname: Rüdiger
authors.forename: Rein
authors.initials: R
authors.lastname: Willemze
authors.forename: Chris J L M
authors.initials: CJ
authors.lastname: Meijer
authors.forename: Emilio
authors.initials: E
authors.lastname: Berti
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cerroni
authors.forename: Marco
authors.initials: M
authors.lastname: Santucci
authors.forename: Christian
authors.initials: C
authors.lastname: Hallermann
authors.forename: Mark
authors.initials: M
authors.lastname: Berneburg
authors.forename: Sergio
authors.initials: S
authors.lastname: Chimenti
authors.forename: Alistair
authors.initials: A
authors.lastname: Robson
authors.forename: Martà
authors.initials: M
authors.lastname: Marschalko
authors.forename: Dmitry V
authors.initials: DV
authors.lastname: Kazakov
authors.forename: Tony
authors.initials: T
authors.lastname: Petrella
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
authors.forename: Agnes
authors.initials: A
authors.lastname: Carlotti
authors.forename: Philippe
authors.initials: P
authors.lastname: Courville
authors.forename: Hubert
authors.initials: H
authors.lastname: Laeng
authors.forename: Robert
authors.initials: R
authors.lastname: Knobler
authors.forename: Philippa
authors.initials: P
authors.lastname: Golling
authors.forename: Reinhard
authors.initials: R
authors.lastname: Dummer
authors.forename: Günter
authors.initials: G
authors.lastname: Burg
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D018450:Disease Progression; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D055815:Young Adult