Novel markers in pediatric-type follicular lymphoma

Claudio Agostinelli, Ayse U. Akarca, Alan Ramsay, Hasan Rizvi, Manuel Rodriguez-Justo, Sabine Pomplun, Ian Proctor, Elena Sabattini, David Linch, Stephen Daw, Stefania Pittaluga, Stefano A. Pileri, Elaine S. Jaffe, Leticia Quintanilla-Martinez, Teresa Marafioti

Research output: Contribution to journalArticlepeer-review


The aim of this study was to review the histopathological, phenotypic, and molecular characteristics of pediatric-type follicular lymphoma (PTFL) and to assess the diagnostic value of novel immunohistochemical markers in distinguishing PTFL from follicular hyperplasia (FH). A total of 13 nodal PTFLs were investigated using immunohistochemistry, fluorescence in situ hybridization (FISH), and PCR and were compared with a further 20 reactive lymph nodes showing FH. Morphologically, PTFL cases exhibited a follicular growth pattern with irregular lymphoid follicles in which the germinal centers were composed of numerous blastoid cells showing a starry-sky appearance. Immunohistochemistry highlighted preserved CD10 (13/13) and BCL6 (13/13) staining, CD20 (13/13) positivity, a K light chain predominance (7/13), and partial BCL2 expression in 6/13 cases (using antibodies 124, E17, and SP66). The germinal center (GC)–associated markers stathmin and LLT-1 were positive in most of the cases (12/13 and 12/13, respectively). Interestingly, FOXP-1 was uniformly positive in PTFL (12/13 cases) in contrast to reactive GCs in FH, where only a few isolated positive cells were observed. FISH revealed no evidence of BCL2, BCL6, or MYC rearrangements in the examined cases. By PCR, clonal immunoglobulin gene rearrangements were detected in 100% of the tested PTFL cases. Our study confirmed the unique morphological and immunophenotypic features of PTFL and suggests that FOXP-1 can represent a novel useful diagnostic marker in the differential diagnosis between PTFL and FH.

Original languageEnglish
Pages (from-to)771-779
Number of pages9
JournalVirchows Archiv
Issue number6
Publication statusPublished - Dec 1 2019


  • Differential diagnosis
  • FOXP-1
  • Immunohistochemistry
  • Pediatric-type follicular lymphoma

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Molecular Biology
  • Cell Biology


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