mantle cell lymphoma
Mantle cell lymphoma (MCL) is characterized by the chromosomal translocation t(11;14)(q13;q32), resulting in overexpression of cyclin-D1 (CCND1) in the vast majority of cases. In addition, alterations of other cell-cycle-regulating signal pathways as CDKN2B/CDKN2A-CCND1 pathway or ARF-MDM2-TP53 pathway are frequently observed.
Immunophenotype
Pan-B +
CD5+
CD23-
CD10-/+
sIgM+ bright
Putative cell of origin: CD5+ B-cells of the follicle mantle having germline IgV gene sequences
Variants
Burkitt lymphoma transformation of mantle cell lymphoma (15370262)
Cyclin D1 and t(11;14)-mantle cell lymphoma resembling marginal zone B-cell lymphoma (17316759)
composite splenic marginal zone lymphoma and mantle cell lymphoma (17134739)
Localization
nodal mantle cell lymphoma
Cytogenetics
translocation t(11;14)(q13;q32) with CCND1 rearrangement with cyclin-D1 overexpression (50-90%) (11q13 and 14q32)
translocation t(1;12)(p21;q23)
complex karyotype
- complex karyotype carries an unfavourable prognostic significance
1p22.3 deletion (116830336)
11q23 deletion (16382446)
Molecular biology
epigenetic alterations and genomic deletions of CDK inhibitors (CDKIs) p16(INK4a), p15(INK4b), p14(ARF) (16258956)
cyclin D1 overexpression by CCND1 gene secondary to the translocation t(11;14)(q13;q32) (11q13 and 14q32)
Reviews
Jares P, Colomer D, Campo E. Genetic and molecular pathogenesis of mantle cell lymphoma: perspectives for new targeted therapeutics. Nat Rev Cancer. 2007 Oct;7(10):750-62. PMID: 17891190
Kurtin PJ. Mantle cell lymphoma. Adv Anat Pathol. 1998 Nov;5(6):376-98. PMID: 10095879
References
Mansoor A, Akbari M, Auer I, Lai R. Cyclin D1 and t(11;14)-positive B-cell neoplasms resembling marginal zone B-cell lymphoma: a morphological variant of mantle cell lymphoma. Hum Pathol. 2007 Feb 19; PMID: 17316759
Balakrishnan A, von Neuhoff N, Rudolph C, Kamphues K, Schraders M, Groenen P, van Krieken JH, Callet-Bauchu E, Schlegelberger B, Steinemann D. Quantitative microsatellite analysis to delineate the commonly deleted region 1p22.3 in mantle cell lymphomas. Genes Chromosomes Cancer. 2006 Oct;45(10):883-92. PMID: 16830336
Hutter G, Scheubner M, Zimmermann Y, Kalla J, Katzenberger T, Hubler K, Roth S, Hiddemann W, Ott G, Dreyling M. Differential effect of epigenetic alterations and genomic deletions of CDK inhibitors [p16(INK4a), p15(INK4b), p14(ARF)] in mantle cell lymphoma. Genes Chromosomes Cancer. 2006 Feb;45(2):203-10. PMID: 16258956