World Health Organization-European Organization For Research And Treatment Of Cancer Research Articles

Subcutaneous panniculitis-like T-cell cutaneous lymphoma

Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a rare cytotoxic T-cell lymphoma classified in the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification as a unique extranodal lymphoma with characteristic by T cell receptor (TCR) gene rearrangement. We report here a case of SPTCL in a 22 year-old woman who had presented with variably sized multiple nodules on both her legs. Initial differential diagnoses considered were panniculitis and lupus panniculitis. The histopathology showed a predominantly subcutaneous lobular infiltrate with atypical lymphocytes, karyorrhexis and rimming of adipocytes by lymphoid cells. Immunohistochemistry showed CD4-, CD8+, CD56- T-cell phenotype. Although TCR rearrangement studies were not done, the above T-cell phenotype and sparing of epidermis and dermis suggested the possibility of an SPTCL alpha/beta type. The patient received five cycles of a cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) regimen which resulted in the regression in her skin lesions and constitutional symptoms.

Results of Radiotherapy in 153 Primary Cutaneous B-Cell Lymphomas Classified According to the WHO-EORTC Classification

To evaluate the results of radiotherapy in patients with primary cutaneous B-cell lymphoma (CBCL) classified according to the criteria of the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification. Multicenter, 20-year, retrospective, cohort analysis. Eight dermatology departments collaborating in the Dutch Cutaneous Lymphoma Group. From 1985 until 2005, a total of 153 patients with CBCL were initially treated with radiotherapy with curative intent. These cases were classified according to the WHO-EORTC classification and consisted of 25 primary cutaneous marginal zone lymphomas (PCMZLs), 101 primary cutaneous follicle center lymphomas (PCFCLs), and 27 primary cutaneous large B-cell lymphomas, leg type (PCLBCLs, LT). Interventions Local radiotherapy with a median dose of 40 Gy (range, 20-46 Gy) applied to all visible skin lesions. Complete remission rate, relapse rate, 5-year relapse-free survival, 5-year overall survival, and 5-year disease-specific survival. Complete remission was reached in 151 of 153 patients (99%). Relapse rates for PCMZL, PCFCL, and PCLBCL, LT were 60%, 29%, and 64%, and the 5-year disease-specific survival was 95%, 97%, and 59%, respectively. The PCFCLs presenting on the legs had a higher relapse rate (63%) and a much lower 5-year disease-specific survival (44%) than PCFCLs at other sites (relapse rate, 25%; 5-year disease-specific survival, 99%). Radiotherapy is a suitable treatment for a large group of patients with CBCL. However, patients with PCFCL presenting with lesions on the leg and patients with PCLBCL, LT display a more unfavorable clinical course and should therefore be treated with more aggressive treatment modalities.

Follikuläres Lymphom mit Übergang in ein diffuses großzelliges B-Zell-Lymphom

Die aktuelle WHO/EORTC-Klassifikation differenziert in der Gruppe der seltenen primär kutanen B-Zell-Lymphome (I) das Marginalzonen B-Zell-Lymphom (PCMZL), (II) das follikuläre Lymphom (PCFCL), und (III) das kutane großzellige B-Zell-Lymphom vom Bein-Typ (PCLBCL-LT) und (IV) andere primär kutane großzellige B-Zell-Lymphome (PCLBCLO) [1]. Letztere sind diffuse großzellige B-Zell-Lymphome, die weder in die Gruppe der PCLBCL-LT noch der PCFCL passen. Während PCFCL und PCLBCL-LT klare Unterschiede in der Klinik, Histopathologie und Prognose zeigen, ist das PCLBCLO durch überlappende klinisch-pathologische Merkmale gekennzeichnet.Ein 47-jähriger Mann bemerkte seit einigen Monaten eine rötliche Makula an der rechten Schulter, die er als Akneeffloreszenz ansah, da er in seiner Jugend unter starker Akne litt. Eine systemische Doxycyclintherapie bei Hinweis auf eine floride Borreliose erbrachte keine Besserung. Eine Hautbiopsie ergab die Diagnose eines follikulären Lymphoms mit Übergang in ein diffuses großzelliges B-Lymphom. Die Therapie mit einer modifizierten R-CHOP (Rituximab, Cyclophosphamid, pegyliertes liposomales Doxorubicin, Vincristin und Prednisolon) zeigte ein gutes Ansprechen.

Extranodal NK/T-Cell Lymphoma Nasal Type Mimicking Pyoderma Gangrenosum

This article does not have an abstract.

Extranodal NK/T-Cell Lymphoma Nasal Type Mimicking Pyoderma Gangrenosum

The new World Health Organization/European Orga-nization for Research and Treatment of Cancer (WHO-EORTC) consensus classification for cutaneous lympho-mas recognizes two main groups of CD56+ lymphomas: extranodal natural killer (NK)/T-cell lymphoma nasal type, and CD56+ haematodermic neoplasm (formerly known as blastic NK cell lymphoma (1). NK/T-cell lymphoma nasal type is the most common lymphoma type among primary lymphomas of the nasal cavity (2).It presents clinically as a nasal mass and may involve the skin secondarily, where it often presents as a mid-facial destructive tumour or as multiple distant erythematous nodules, which can evolve to ulceration (3). There is a strong association with Epstein-Barr virus (4).We report here a patient with an extranodal NK/T-cell lymphoma nasal type with cutaneous involvement, in which the clinical presentation mimicked pyoderma gangrenosum. CASE REPORT

Prognosis of Lymphomatoid Papulosis

Correspondence: Matthias Schmuth, M.D., Department of Dermatology, Anichstrase 35, A-6020 Innsbruck, Austria. Telephone: 43-512504-81472; Fax: 43-512-504-22990; e-mail: matthias.schmuth@uibk.ac.at Received May 15, 2006; accepted July 10, 2006. ©AlphaMed Press 1083-7159/2006/$20.00/0 doi: 10.1634/theoncologist.11-8-955 Because of its characteristic waxing and waning course, lymphomatoid papulosis (LyP) was previously considered a pseudolymphomatous inflammatory process. However the recent World Health Organization–European Organization for Research and Treatment of Cancer (WHO-EORTC) classification grouped LyP among the indolent cutaneous lymphomas [1]. The rationale for classifying LyP as a cutaneous lymphoma is its association with other lymphoproliferative disorders, particularly the progression of LyP into frank lymphoma, that is, Hodgkin’s disease, cutaneous Tcell lymphoma, or anaplastic large cell lymphoma. In their article “Clinical Mimics of Lymphoma,” Brown and Skarin [2] report a rate of progression of LyP to frank lymphoma of 20%–80%. The high variability in these numbers prompted us to reassess the rate of progression in our patient cohort and in cases reported in the literature, in particular because of the therapeutic consequences involved. Although it seems clear that undue aggressive treatment must be avoided, a high advancing rate of the disorder should entail a therapeutic approach aimed at halting the disease process. In contrast, a low rate of progression would be in favor of a “wait and see” approach. A retrospective analysis of our cohort (11 female, 10 male; median age, 47 years; range, 6–77 years) revealed that the absolute frequency of LyP preceding lymphoma was 2 of 21 patients (9.5%) (Table 1). In comparison, absolute frequencies between 5% and 24% have been reported in the literature [3–10] (Table 2). However, rather than calculating the absolute frequency in a given LyP patient cohort, an analysis of the course of the disease indicates that there is a considerably increased risk for progression when LyP is followed up for extended time periods (Table 3). Therefore, the cumulative risk for progression over time may represent a more relevant basis for therapeutic decisions. Yet estimates of the cumulative risk for progression of LyP to lymphoma vary greatly in the literature. For instance, Bekkenk et al. [7] reported cumulative risks in a cohort of 118 LyP patients of 2%, 4%, and 12% at 5, 10, and 15 years after

Linfoma NK blástico

Blastic NK («natural killer») cell lymphoma is a rare type of lymphoma, recognized as an independent entity in the new World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for cutaneous lymphomas. Its clinical, morphological and immunophenotypical characteristics are relatively well defined. It frequently starts with extranodal involvement (primarily cutaneous), often presents with splenomegaly and pancytopenia, and initially responds well to chemotherapy, although the medium-term prognosis is usually poor. It was first considered to originate from immature NK lymphocytes, but now its origin is thought to be plasmacytoid dendritic cell precursors. We present the clinicopathological characteristics of a new case of this entity in a 78-year-old male patient.

Primary cutaneous large B-cell lymphomas: clinicopathologic features, classification, and prognostic factors in a large series of patients

AbstractIn the new World Health Organization/European Organization for Research and Treatment of Cancer (WHO/EORTC) classification of cutaneous lymphomas, large B-cell lymphomas (LBCLs) are divided into 3 groups: LBCL, leg-type (LBCLLT); follicle center lymphoma, diffuse type (FCLDT); and LBCL, others (LBCLO). We studied a large number of primary cutaneous LBCLs to test the validity of the classification and to identify prognostic factors for these patients. Ninety-three cases of primary cutaneous LBCL were analyzed for clinicopathologic features, expression of several markers including Bcl-2, Bcl-6, MUM-1, and FOX-P1, in situ hybridization for Epstein-Barr virus, and molecular analyses of IGH gene rearrangement and of Borrelia burgdorferi and human herpesvirus 8 DNA. Patients were classified into the following categories: FCLDT, 44 cases; LBCLLT, 40 cases; and LBCLO, 9 cases. Statistical analyses showed that the LBCLLT and FCLDT groups were clearly distinct in terms of clinicopathologic features and survival. The LBCLO group had features in between those of LBCLLT and FCLDT. Our study shows that accurate morphologic and phenotypic analyses allow us to stratify most patients into the prognostically different categories of LBCLLT and FCLDT. The definition of a third category of LBCLO requires further studies to clarify whether these cases indeed show distinct clinicopathologic features. (Blood. 2005;106:2491-2497)

The new World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas: a practical marriage of two giants

Following consensus meetings of the two parent organizations, a new World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for primary cutaneous lymphomas has recently been published. This important development will now end the ongoing debate as to which of these was the preferred classification. The new classification will facilitate more uniformity in diagnosis, management and treatment of cutaneous lymphomas. In particular, it provides a useful distinction between indolent and more aggressive types of primary cutaneous lymphoma and provides practical advice on preferred management and treatment regimens. This will thereby prevent patients receiving high-grade treatment for low-grade biological disease. This review focuses on those diseases which have found new consensus agreement compared with the original WHO and EORTC classifications. In cutaneous T-cell lymphomas, these include folliculotropic mycosis fungoides, defining features of Sézary syndrome, primary cutaneous CD30+ lymphoproliferative disorders (primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis and borderline lesions) and subcutaneous panniculitis-like T-cell lymphoma. Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma, primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma and cutaneous gamma/delta T-cell lymphoma are allocated provisional entry status and thereby afford better definitions for some cases of currently unspecified primary cutaneous peripheral T-cell lymphoma. In cutaneous B-cell lymphomas, diseases which have found new consensus agreement include primary cutaneous marginal zone B-cell lymphoma, primary cutaneous follicular centre lymphoma, primary cutaneous diffuse large B-cell lymphoma, leg type and primary cutaneous diffuse large B-cell lymphoma, other. CD4+/CD56+ haematodermic neoplasm (early plasmacytoid dendritic cell leukaemia/lymphoma) now appears as a precursor haematological neoplasm and replaces the previous terminology of blastic NK-cell lymphoma. Other haematopoietic and lymphoid tumours involving the skin, as part of systemic disease, will appear in the forthcoming WHO publication Tumours of the Skin. The new classification raises interesting new problems and questions about primary cutaneous lymphoma and some of these are discussed in this article. It is, however, a splendid signpost indicating the direction in which research in cutaneous lymphoma needs to go. In the interim, we have an international consensus classification which is clinically meaningful.