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Clinicopathologic Features and Prognostic Analysis in 60 Cases

Florent Grange, MD, PhD; Marie Beylot-Barry, MD, PhD; Phillipe Courville, MD; Eve Maubec, MD; Martine Bagot, MD, PhD; Béatrice Vergier, MD, PhD; Pierre Souteyrand, MD; Laurent Machet, MD, PhD; Sophie Dalac, MD; Eric Esteve, MD; Isabelle Templier, MD; Emmanuel Delaporte, MD; Marie-Françoise Avril, MD; Caroline Robert, MD, PhD; Stephane Dalle, MD; Liliane Laroche, MD, PhD; Michele Delaunay, MD; Pascal Joly, MD, PhD; Janine Wechsler, MD; Tony Petrella, MD

Arch Dermatol. 2007;143(9):1144-1150.

Objectives  To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization–European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas.

Design  Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas.

Setting  Nineteen departments of dermatology in 10 regions of France.

Patients  Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas.

Main Outcome Measures  Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Disease-specific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model.

Results  Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable short-term outcome.

Conclusions  Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.

Author Affiliations: Department of Dermatology, Hôpital Robert Debré, Reims (Dr Grange); Departments of Dermatology (Dr Beylot-Barry) and Pathology (Dr Vergier), Hôpital du Haut Lévêque, Pessac; Departments of Dermatology (Dr Courville) and Pathology (Dr Joly), Hôpital Charles Nicolle, Rouen; Department of Dermatology, Hôpital Bichat (Dr Maubec), and Department of Dermatology, Hôpital Tarnier (Dr Avril), Paris; Departments of Dermatology (Dr Bagot) and Pathology (Dr Wechsler), Hôpital Henri-Mondor, Créteil; Department of Dermatology, Hôtel Dieu, Clermont-Ferrand (Dr Souteyrand); Department of Dermatology, Hôpital Trousseau, Tours (Dr Machet); Department of Dermatology (Dr Dalac) and Centre de Pathologie and Department of Pathology (Dr Petrella), Hôpital du Bocage, Dijon; Department of Dermatology, Hôpital Porte Madeleine, Orléans (Dr Esteve); Department of Dermatology, Hôpital Michallon, Grenoble (Dr Templier); Department of Dermatology, Hôpital Claude Huriez, Lille (Dr Delaporte); Department of Dermatology, Institut Gustave Roussy, Villejuif (Drs Avril and Robert); Department of Dermatology, Hôpital de l’Hôtel-Dieu, Lyon (Dr Dalle); Department of Dermatology, Hôpital Avicenne, Bobigny (Dr Laroche); and Department of Dermatology and Cancerology, Hôpital Pellegrin, Bordeaux (Dr Delaunay); France.

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