Molecular Pathogenesis of Mantle Cell Lymphoma

Hematol Oncol Clin North Am. 2020 Oct;34(5):795-807. doi: 10.1016/j.hoc.2020.05.002. Epub 2020 Jul 22.

Abstract

Mantle cell lymphoma (MCL) is a mature B-cell neoplasm with heterogeneous clinical behavior molecularly characterized by the constitutive overexpression of cyclin D1 and deregulation of different signaling pathways. SOX11 expression determines an aggressive phenotype associated with accumulation of many chromosomal alterations and somatic gene mutations. A subset of patients with the SOX11-negative leukemic non-nodal MCL subtype follows an initial indolent clinical evolution and may not require treatment at diagnosis, although eventually may progress to an aggressive disease. We discuss the genetic and molecular alterations with impact on the cancer hallmarks that characterize the lymphomagenesis of the 2 MCL subtypes.

Keywords: CCND1; Cancer hallmarks; Gene mutations; Genome instability; Mantle cell lymphoma; Molecular pathogenesis; SOX11.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Cyclin D1* / biosynthesis
  • Cyclin D1* / genetics
  • Gene Expression Regulation, Neoplastic*
  • Humans
  • Lymphoma, Mantle-Cell* / classification
  • Lymphoma, Mantle-Cell* / genetics
  • Lymphoma, Mantle-Cell* / metabolism
  • Lymphoma, Mantle-Cell* / pathology
  • Mutation*
  • SOXC Transcription Factors* / biosynthesis
  • SOXC Transcription Factors* / genetics

Substances

  • CCND1 protein, human
  • SOX11 protein, human
  • SOXC Transcription Factors
  • Cyclin D1