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A Multicenter Study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization for Research and Treatment of Cancer (EORTC)

Werner Kempf, MD; Sonja Ostheeren-Michaelis, MD; Marco Paulli, MD; Marco Lucioni, MD; Janine Wechsler, MD; Heike Audring, MD; Chalid Assaf, MD; Thomas Rüdiger, MD; Rein Willemze, MD; Chris J. L. M. Meijer, MD; Emilio Berti, MD; Lorenzo Cerroni, MD; Marco Santucci, MD; Christian Hallermann, MD; Mark Berneburg, MD; Sergio Chimenti, MD; Alistair Robson, MBBS; Martà Marschalko, MD; Dmitry V. Kazakov, MD, PhD; Tony Petrella, MD; Sylvie Fraitag, MD; Agnes Carlotti, MD; Philippe Courville, MD; Hubert Laeng, MD; Robert Knobler, MD; Philippa Golling, MD; Reinhard Dummer, MD; Günter Burg, MD

Arch Dermatol. 2008;144(12):1609-1617.

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.

Author Affiliations: Departments of Dermatology, University Hospital, Zürich, Switzerland (Drs Kempf, Ostheeren-Michaelis, Golling, Dummer, and Burg), Hôpital Henri Mondor, Creteil, France (Dr Wechsler), Charité, Berlin, Germany (Drs Audring and Assaf), Leiden University, Medical Center, Leiden, the Netherlands (Dr Willemze), University Milan-Bicocca, Milan, Italy (Dr Berti), Medical University of Graz, Graz, Austria (Dr Cerroni), University Hospital Tübingen, Tübingen, Germany (Dr Berneburg), University of Rome, Rome, Italy (Dr Chimenti), Semmelweis Medical School, Budapest, Hungary (Dr Marschalko), Necker-Enfants Malades Hopital, Paris, France (Dr Fraitag), and Hopital Tarnier Cochin, Paris, France (Dr Carlotti); Departments of Pathology, University of Pavia, Pavia, Italy (Drs Paulli and Lucioni), Hôpital Henri Mondor, Creteil, France (Dr Wechsler), Luitpold Hospital, Würzburg, Germany (Dr Rüdiger), Vrije Universiteit Medical Center, Amsterdam, the Netherlands (Dr Meijer), University Hospital, Djion, France (Drs Petrella and Courville), and Cantonal Hospital, Aarau, Switzerland (Dr Laeng); Department of Human Pathology and Oncology, University of Florence Medical School, Florence, Italy (Dr Santucci); Hautklinik Linden, Hannover, Germany (Dr Hallermann); Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, London, England (Dr Robson); Sikl's Department of Pathology, Charles University, Medical Faculty Hospital, Pilsen, Czech Republic (Dr Kazakov); and Division of Special and Environmental Dermatology, Department of Dermatology, Vienna, Austria (Dr Knobler).

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RELATED LETTERS

"Cutaneous T-Cell Lymphoma": Cutaneous TNMania in Cutaneous Lymphomas
Peter Heald
Arch Dermatol. 2009;145(7):842-843.
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"Cutaneous T-Cell Lymphoma": Cutaneous TNMania in Cutaneous Lymphomas—Reply
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Arch Dermatol. 2009;145(7):843.
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