title: Aberrant nuclear BCL10 expression and lack of t(11;18)(q21;q21) in primary cutaneous marginal zone B-cell lymphoma.
abstract: Inhibition of apoptosis seems to play an important role in the pathogenesis of marginal zone lymphoma. Apoptosis regulator B-cell lymphoma 10 (BCL10) may show aberrant nuclear localization in some aggressive extracutaneous MALT lymphomas, often in association with a MALT1 gene t(11;18)(q21;q21) translocation. The possible occurrence of this association in primary cutaneous marginal zone lymphoma (PCMZL) remains insufficiently explored. The aim of this study was to evaluate BCL10 protein expression pattern and its possible relationship to the presence of t(11;18)(q21;q21) and other MALT1 gene abnormalities in PCMZL and to assess their clinical significance. The study included 42 consecutive PCMZL patients diagnosed on the basis of the World Health Organization/European Organization for the Research and Treatment of Cancer classification criteria. BCL10 expression was immunohistochemically evaluated in all cases, whereas t(11;18)(q21;q21) reverse transcriptase polymerase chain reaction amplification was performed on 21 samples. In addition, the presence of other MALT1 gene translocations was explored in 26 samples by interphase fluorescence in situ hybridization using a MALT1 locus-specific probe. We observed the presence of aberrant nuclear BCL10 expression in a significant number of PCMZL cases (36%, 15/42). This aberrant expression was significantly related to the development of extracutaneous disease. In contrast, neither the t(11;18)(q21;q21) translocation nor other MALT1 gene translocations could be demonstrated. t(11;18)(q21;q21), strongly linked to extracutaneous MALT lymphomas, does not seem to play a role in PCMZL. The participation of other MALT1 gene translocations in PCMZL pathogenesis seems also unlikely.
authors.affiliation: Department of Dermatology, Hospital del Mar, IMAS, Barcelona, Spain; Catalonian Cutaneous Lymphoma Network, Barcelona, Spain. fgallardo@imas.imim.es
authors.forename: Fernando
authors.initials: F
authors.lastname: Gallardo
authors.forename: Beatriz
authors.initials: B
authors.lastname: Bellosillo
authors.forename: Blanca
authors.initials: B
authors.lastname: Espinet
authors.forename: Ramon M
authors.initials: RM
authors.lastname: Pujol
authors.forename: Teresa
authors.initials: T
authors.lastname: Estrach
authors.forename: Octavio
authors.initials: O
authors.lastname: Servitje
authors.forename: Vicenç
authors.initials: V
authors.lastname: Romagosa
authors.forename: Carles
authors.initials: C
authors.lastname: Barranco
authors.forename: Susana
authors.initials: S
authors.lastname: Boluda
authors.forename: Mar
authors.initials: M
authors.lastname: García
authors.forename: Francesc
authors.initials: F
authors.lastname: Solé
authors.forename: Aurelio
authors.initials: A
authors.lastname: Ariza
authors.forename: Sergi
authors.initials: S
authors.lastname: Serrano
mesh_terms: D048868:Adaptor Proteins, Signal Transducing / Q000096:biosynthesis*; D000368:Aged; D000074018:B-Cell CLL-Lymphoma 10 Protein; D002467:Cell Nucleus / Q000378:metabolism; D002880:Chromosomes, Human, Pair 11; D002883:Chromosomes, Human, Pair 14; D005260:Female; D049669:History, 16th Century; D049670:History, 17th Century; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics* / Q000378:metabolism* / Q000401:mortality; D008297:Male; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012878:Skin Neoplasms / Q000235:genetics* / Q000378:metabolism* / Q000401:mortality; D016019:Survival Analysis; D014178:Translocation, Genetic
chemical_list: D048868:Adaptor Proteins, Signal Transducing; D000074018:B-Cell CLL-Lymphoma 10 Protein; C118591:BCL10 protein, human