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).
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.
Kempf
Werner
W
Department of Dermatology, University Hospital Zürich, Gloriastrasse 31, CH-8091 Zürich, Switzerland. kempf@derm.unizh.ch.
Ostheeren-Michaelis
Sonja
S
Paulli
Marco
M
Lucioni
Marco
M
Wechsler
Janine
J
Audring
Heike
H
Assaf
Chalid
C
Rüdiger
Thomas
T
Willemze
Rein
R
Meijer
Chris J L M
CJ
Berti
Emilio
E
Cerroni
Lorenzo
L
Santucci
Marco
M
Hallermann
Christian
C
Berneburg
Mark
M
Chimenti
Sergio
S
Robson
Alistair
A
Marschalko
Martà
M
Kazakov
Dmitry V
DV
Petrella
Tony
T
Fraitag
Sylvie
S
Carlotti
Agnes
A
Courville
Philippe
P
Laeng
Hubert
H
Knobler
Robert
R
Golling
Philippa
P
Dummer
Reinhard
R
Burg
Günter
G
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