Role of SOX11 and Genetic Events Cooperating with Cyclin D1 in Mantle Cell Lymphoma.

ABSTRACT

Mantle cell lymphoma (MCL) is an aggressive lymphoid neoplasm, incurable with current therapies. The t(11;14)(q13;q32) involving cyclin D1 is considered the first oncogenic hit found in virtually all MCLs. However, additional secondary genomic alterations are essential for complete transformation. MCLs are genetically very unstable with several genetic alterations associated with its high proliferative behavior involving several oncogenic pathways. Furthermore, SOX11 is overexpressed in the majority of conventional MCLs (cMCL), including cyclin D1-negative cases, but absent in non-nodal leukemic MCL with indolent clinical behavior (nnMCL). Recent data have revealed the potential oncogenic role of SOX11 in MCL biology, highlighting its implication in tumor aggressiveness and progression. This review addresses the implication of SOX11 overexpression and frequent genetic lesions, cooperating with cyclin D1 underlying the pathogenesis of this aggressive disease.