TY - JOUR
T1 - Granulomatous mycosis fungoides and granulomatous slack skin
T2 - A multicenter study of the cutaneous lymphoma histopathology task force group of the European Organization for Research and Treatment of Cancer (EORTC)
AU - Kempf, Werner
AU - Ostheeren-Michaelis, Sonja
AU - Paulli, Marco
AU - Lucioni, Marco
AU - Wechsler, Janine
AU - Audring, Heike
AU - Assaf, Chalid
AU - Rüdiger, Thomas
AU - Willemze, Rein
AU - Meijer, Chris J L M
AU - Berti, Emilio
AU - Cerroni, Lorenzo
AU - Santucci, Marco
AU - Hallermann, Christian
AU - Berneburg, Mark
AU - Chimenti, Sergio
AU - Robson, Alistair
AU - Marschalko, Martà
AU - Kazakov, Dmitry V.
AU - Petrella, Tony
AU - Fraitag, Sylvie
AU - Carlotti, Agnes
AU - Courville, Philippe
AU - Laeng, Hubert
AU - Knobler, Robert
AU - Golling, Philippa
AU - Dummer, Reinhard
AU - Burg, Günter
PY - 2008/12
Y1 - 2008/12
N2 - 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. Observations: 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.
AB - 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. Observations: 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.
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U2 - 10.1001/archdermatol.2008.46
DO - 10.1001/archdermatol.2008.46
M3 - Article
C2 - 19075143
AN - SCOPUS:58049195512
VL - 144
SP - 1609
EP - 1617
JO - Archives of Dermatology
JF - Archives of Dermatology
SN - 0003-987X
IS - 12
ER -