Abstract
Mantle cell lymphoma is a B cell malignancy in which constitutive dysregulation of cyclin D1 and the cell cycle, disruption of DNA damage response pathways, and activation of cell survival mechanisms contribute to oncogenesis. A small number of tumors lack cyclin D1 overexpression, suggesting that its dysregulation is always not required for tumor initiation. Some cases have hypermutated IGHV and stable karyotypes, a predominant nonnodal disease, and an indolent clinical evolution, which suggests that they may correspond to distinct subtypes of the disease. In this review, we discuss the molecular pathways that contribute to pathogenesis, and how improved understanding of these molecular mechanisms offers new perspectives for the treatment of patients.
Publication types
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Apoptosis / genetics
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B-Lymphocytes / pathology
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Cell Transformation, Neoplastic / genetics*
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Chromosomes, Human, Pair 11 / genetics*
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Chromosomes, Human, Pair 11 / ultrastructure
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Chromosomes, Human, Pair 14 / genetics*
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Chromosomes, Human, Pair 14 / ultrastructure
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Clone Cells / pathology
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Complementarity Determining Regions / genetics
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Cyclin D1 / biosynthesis
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Cyclin D1 / physiology
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DNA Repair / genetics
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Disease Progression
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Gene Rearrangement, B-Lymphocyte, Heavy Chain
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Genes, Immunoglobulin
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Genes, bcl-1
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Germinal Center / pathology
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Humans
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Immunoglobulin Heavy Chains / genetics
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Lymphoma, Mantle-Cell / drug therapy
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Lymphoma, Mantle-Cell / etiology
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Lymphoma, Mantle-Cell / genetics*
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Lymphoma, Mantle-Cell / pathology
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Molecular Targeted Therapy
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Neoplasm Invasiveness
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Neoplasm Proteins / biosynthesis
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Neoplasm Proteins / genetics*
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Neoplasm Proteins / physiology
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Neoplastic Stem Cells / pathology
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Signal Transduction / genetics
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Signal Transduction / physiology
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Stem Cell Niche
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Translocation, Genetic*
Substances
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CCND1 protein, human
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Complementarity Determining Regions
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Immunoglobulin Heavy Chains
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Neoplasm Proteins
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Cyclin D1