The pathologic diagnosis of mantle cell lymphoma

Histol Histopathol. 2021 Oct;36(10):1037-1051. doi: 10.14670/HH-18-351. Epub 2021 Jun 11.

Abstract

Mantle cell lymphoma (MCL) is a mature B-cell non-Hodgkin lymphoma usually characterized by t(11;14) (q13;q32), or CCND1 translocation and Cyclin D1 over expression. A very small subset of MCL may lack the t(11;14) (q13;q32) translocation and Cyclin D1 over expression, but show alternative translocations involving CCND2 and CCND3, and over expression of SOX11. In general, MCL has been considered a very aggressive and incurable lymphoma and patients with MCL usually have a poor prognosis. However, indolent variants, including in situ mantle cell neoplasm and the recently recognized leukemic non-nodal MCL do exist. In recent years, genome-wide molecular genetic studies have revealed a characteristic MCL genetic profile. This review will focus on the pathologic diagnosis of MCL using the traditional morphological and immunophenotypic strategies combined with cytogenetic characteristics and recently identified molecular profile. Morphological subtypes, immunophenotypic variants, recently recognized indolent variants, as well as MCL risk stratification will also be discussed.

Publication types

  • Review

MeSH terms

  • Cyclin D1 / genetics
  • Cyclin D2 / genetics
  • Cyclin D3 / genetics
  • Genome-Wide Association Study
  • Humans
  • Immunohistochemistry
  • Lymphoma, Mantle-Cell / diagnosis*
  • Lymphoma, Mantle-Cell / genetics
  • Lymphoma, Mantle-Cell / pathology*
  • Translocation, Genetic

Substances

  • CCND1 protein, human
  • CCND2 protein, human
  • CCND3 protein, human
  • Cyclin D2
  • Cyclin D3
  • Cyclin D1