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Pleomorphic mantle cell lymphoma morphologically mimicking diffuse large B cell lymphoma: common cyclin D1 negativity

Wen-Yu Chuang1,2,3, Hung Chang4, Gwo-Jyh Chang2

  • 1Department of Pathology, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Taoyuan, Taiwan.

Histopathology
|December 30, 2016
PubMed

Insights

Pleomorphic mantle cell lymphoma (PMCL) can mimic diffuse large B cell lymphoma (DLBCL). A simple immunohistochemical algorithm helps identify PMCL, even when cyclin D1 negative, preventing misdiagnosis and ensuring appropriate treatment for this aggressive lymphoma.

Area of Science:

  • Hematopathology
  • Oncology
  • Genetics

Background:

  • Pleomorphic mantle cell lymphoma (PMCL) is a rare variant that morphologically resembles diffuse large B cell lymphoma (DLBCL).
  • Accurate diagnosis is crucial for appropriate treatment and patient outcomes.

Purpose of the Study:

  • To characterize the clinicopathological and genetic features of PMCL.
  • To identify diagnostic markers for PMCL that mimics DLBCL.
  • To investigate the role of cyclin D1 negativity in PMCL.

Main Methods:

  • Systematic screening of 500 B cell lymphomas using an immunohistochemical algorithm (CD5, cyclin D1, SOX11).
  • Identification and further study of 10 PMCL cases.
  • Genetic analysis including IGH-CCND1 translocation, CCND2 translocation, CCND2 mRNA levels, and genomewide copy number profiling.

Main Results:

  • Ten PMCL cases were identified, with 40% unexpectedly negative for cyclin D1.
  • Cyclin D1-negative PMCL cases showed similar genomic profiles to classical mantle cell lymphoma (MCL).
  • CCND2 translocation and copy number gains in PIK3CA and CCDC50 were observed in cyclin D1-negative PMCL, suggesting alternative oncogenic pathways.

Conclusions:

  • Cyclin D1 negativity is surprisingly common in PMCL mimicking DLBCL.
  • A simple immunohistochemical algorithm is effective in preventing misclassification of PMCL.
  • Understanding these features is vital for accurate diagnosis and treatment of PMCL.
Abstract

Keywords:
Mantle cell lymphomacopy number analysiscyclin D1diffuse large B cell lymphomapleomorphic variant

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