Humpath.com - Human pathology

Home > G. Tumoral pathology > mantle cell lymphoma

mantle cell lymphoma

Tuesday 30 September 2003

small cell mantle lymphoma

Definition: 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.

Images

- Mantle cell lymphoma with prominent nodular pattern

Digital cases

- HPC:286 : Nodal mantle cell lymphoma
- HPC:301 : Mantle cell lymphoma of the ocular conjunctiva
- HPC:345 : Mantle cell lymphoma (lymph node)
- HPC:353 : Mantle cell lymphoma (lymph node)

The variable morphology of mantle cell lymphoma (MCL) is assumed to reflect progression from an early form with classical cytology and mantle zone or nodular growth to a later, more aggressive variant of the disease with blastoid cytology and a diffuse growth pattern. (23240716)

In the majority of cases, the mantle zone growth pattern is lost, but it had been reacquired in a small subset of MCLs at relapse. (23240716)

Twenty-two per cent of MCLs with classical/small cell cytology acquire blastoid features during the course of the disease. (23240716)

However, 50% of MCLs with blastoid cytology at primary diagnosis recurred as a classical variant. (23240716)

Ki67

- Ki67

  • The Ki67 index increases over time, and is associated with prognosis in the primary and the relapse biopsy specimens. (23240716)
  • In a large cohort of MCLs, it has been showed that the Ki67 index increases over time in MCL. (23240716)
  • Assessment of the Ki67 index is a useful prognostic tool if assessment is performed in the relapse situation. (23240716)

Immunophenotype

- CD20+, CD5+, CD23-, CD10-, BCL6-
- sIgM+ bright
- Pan-B + (CD20, CD19)
- Positive; BCL2, CyD1
- Usually positive: CD5+, FMC-7, CD43
- Negative: CD10-, BCL6-, CD23-
- CD10-/+
- Aberrant phenotype: CD5-, CD10+, BCL6+

Putative cell of origin: CD5+ B-cells of the follicle mantle having germline IgV gene sequences.

Differential diagnosis

- diffuse small B-cell lymphomas

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

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.

- 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

In addition, alterations of other cell-cycle-regulating signal pathways as CDKN2B/CDKN2A-CCND1 pathway or ARF-MDM2-TP53 pathway are frequently observed.

- 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)

See also

- B-cell lymphomas

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

- Variability in morphology and cell proliferation in sequential biopsies of mantle cell lymphoma at diagnosis and relapse: clinical correlation and insights into disease progression. Vogt N, Klapper W. Histopathology. 2013 Jan;62(2):334-42. doi : 10.1111/his.12009 PMID: 23240716

- Pathway discovery in mantle cell lymphoma by integrated analysis of high resolution gene expression and copy number profiling. Hartmann EM, Campo E, Wright G, Lenz G, Salaverria I, Jares P, Xiao W, Braziel RM, Rimsza LM, Chan WC, Weisenburger DD, Delabie J, Jaffe ES, Gascoyne RD, Dave SS, Mueller-Hermelink HK, Staudt LM, Ott G, BeĆ  S, Rosenwald A. Blood. 2010 Apr 26. PMID: 20421449

- 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

Portfolio