RESUMO
The aim of this study was retrospectively to assess the validity of the 2005 WHO-EORTC classification for primary cutaneous lymphomas (PCL) in a large cohort of patients of a single German skin cancer unit. All patients with PCLs consecutively visiting our hospital between January 1980 and December 2005 were included in a retrospective monocentre study, analysing their histological and clinical data. A total of 312 patients fulfilled the inclusion criteria for PCL. In 299 patients clinical information and paraffin material were sufficient for detailed classification. Of the 299 patients, 63% expressed a T-cell and 37% a B-cell phenotype. Mycosis fungoides was the entity with the highest frequency (30.9%), followed by primary cutaneous follicle centre lymphomas (16.9%) and lymphomatoid papulosis (15.9%). The mean follow-up period was 38.4 months. Five-year disease-specific survival was 80.5% for mycosis fungoides, 92.5% in primary cutaneous anaplastic large cell lymphoma, 100% in lymphomatoid papulosis, 98.1% in primary cutaneous follicle center lymphoma, 100% in primary cutaneous marginal zone lymphoma and 63.2% in diffuse large B-cell lymphoma, leg type. Our data are in line with the data collected by the WHO-EORTC. This is further evidence for the reliability of the WHO-EORTC classification and staging system.
Assuntos
Linfoma de Células B/diagnóstico , Linfoma de Células B/mortalidade , Linfoma Cutâneo de Células T/diagnóstico , Linfoma Cutâneo de Células T/mortalidade , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/mortalidade , Adulto , Idoso , Análise de Variância , Distribuição de Qui-Quadrado , Intervalo Livre de Doença , Feminino , Alemanha/epidemiologia , Humanos , Estimativa de Kaplan-Meier , Linfoma de Células B/classificação , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/mortalidade , Linfoma Difuso de Grandes Células B/diagnóstico , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Cutâneo de Células T/classificação , Papulose Linfomatoide/diagnóstico , Papulose Linfomatoide/mortalidade , Masculino , Pessoa de Meia-Idade , Micose Fungoide/diagnóstico , Micose Fungoide/mortalidade , Estadiamento de Neoplasias , Valor Preditivo dos Testes , Prognóstico , Modelos de Riscos Proporcionais , Reprodutibilidade dos Testes , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Neoplasias Cutâneas/classificação , Taxa de Sobrevida , Fatores de TempoRESUMO
BACKGROUND: There is a growing body of literature that has enhanced our understanding of the biology of primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) including in the context of gene profiling studies. Recent studies have demonstrated an activated proliferation profile associated with leg type lymphoma including overexpression of proto-oncogenes PIM1, PIM2, and cMYC, and the transcription factors MUM1 and OCT2. Although gene profiling is very useful in understanding the molecular basis of diffuse large B-cell lymphoma (LBCL), it is not practical from a routine diagnostic perspective. In this regard, the purpose of the study was to further define an armamentarium of easily applied immunohistochemical stains to accurately prognosticate PCDLBCL. METHODS: In all, 35 patients with PCDLBCL, 14 of follicle center and 21 of leg type, were analyzed using antibodies against CD5, CD138, BCL2, BCL6, OCT2, MUM1, FOXP1, and cMYC. Findings were correlated with clinical data. RESULTS: All cases stained negative for CD5 and CD138. Both subtypes differed in distinct staining patterns for BCL6, BCL2, OCT2, MUM1, and FOXP1. Staining for BCL2, OCT2, and/or MUM1 was associated with poor, and BCL6 with a favorable prognosis. Expression of cMYC was irrespective of prognosis or subtype, whereas ulceration or primary manifestation on the leg or multiple lesions was indicative for worse prognosis. LIMITATIONS: Case number was a limitation. CONCLUSION: Discriminating PCDLBCL supports the validity of the World Health Organization/European Organization for Research and Treatment of Cancer classification. To identify risk factors in patients with PCDLBCL we recommend thorough evaluation of clinical presentation and exploratory staining pattern for BCL2, BCL6, MUM1 and OCT2.
Assuntos
Biomarcadores Tumorais/análise , Genes bcl-2/genética , Linfoma Difuso de Grandes Células B/patologia , Transportador 1 de Cátions Orgânicos/metabolismo , Neoplasias Cutâneas/patologia , Sindecana-1/metabolismo , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Biópsia por Agulha , Estudos de Coortes , Terapia Combinada , Feminino , Humanos , Imuno-Histoquímica , Linfoma Difuso de Grandes Células B/mortalidade , Linfoma Difuso de Grandes Células B/terapia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Transportador 1 de Cátions Orgânicos/genética , Probabilidade , Prognóstico , Modelos de Riscos Proporcionais , Fatores de Risco , Estudos de Amostragem , Sensibilidade e Especificidade , Fatores Sexuais , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/terapia , Análise de Sobrevida , Sindecana-1/genética , Técnicas de Cultura de TecidosRESUMO
INTRODUCTION: Tumor cells of primary cutaneous T-cell lymphomas are able to adopt a regulatory T-cell phenotype in vitro. The significance of this finding in vivo is matter of debate. METHODS: We stained five cases with transformed mycosis fungoides (MF) with an antibody against FOXP3, which is a sensitive and specific marker for the regulatory T-cell phenotype. RESULTS: Transformed T cells in four of five patients with MF stained positive for FOXP3. One patient who showed no CD30 expression of large transformed T cells was also negative for FOXP3. Comparison of plaques and tumors in one patient showed that FOXP3 and CD30 expression was exclusively observed in large transformed tumor cells whereas malignant T cells without large cell transformation were negative. CONCLUSION: Transformation of MF to high grade lymphoma may be associated with the adoption of a regulatory T-cell phenotype. FOXP3 expression may contribute to aggressive behavior of MF after large cell transformation via immune escape mechanism. The significance of this observation is limited by the low case number in this study.