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Primary follicular lymphoma of the testis in childhood: an entity with peculiar clinical and molecular characteristics
  1. S A Pileri1,
  2. E Sabattini1,
  3. P Rosito2,
  4. P L Zinzani1,
  5. S Ascani1,
  6. G Fraternali-Orcioni1,
  7. B Gamberi1,
  8. M Piccioli1,
  9. D Vivenza3,
  10. B Falini4,
  11. G Gaidano3
  1. 1Pathology and Clinical Units of the Institute of Haematology and Clinical Oncology “L. and A. Seràgnoli”, University of Bologna, 40138 Bologna, Italy
  2. 2Unit of Paediatric Haematology and Oncology, Department of Paediatrics, University of Bologna
  3. 3Division of Internal Medicine, Department of Medical Sciences, University of Eastern Piedmont “Amedeo Avogadro”, 28100 Novara, Italy
  4. 4Haematopathology Laboratory, Institute of Haematology, University of Perugia, 06100 Perugia, Italy
  1. Correspondence to:
 Professor S A Pileri, Terza Cattedra di Anatomia Patologica, Istituto di Ematologia ed Oncologia Medica “L. e A. Seràgnoli”, Università di Bologna, Policlinico S. Orsola, Via Massarenti 9, 40138 Bologna, Italy;


Background/Aims: Paediatric primary follicular lymphoma of the testis (PPFLT) is exceptional: the few reported cases seem to lack BCL-2 gene rearrangement and/or protein expression. The aim of this study was to characterise a PPFLT arising in a 4 year old boy.

Methods: This case was characterised using conventional histological analysis, immunohistochemistry, and a polymerase chain reaction based method for the detection of immunoglobulin VH chain rearrangements.

Results: The neoplasm was staged IE/A; left orchiectomy and chemotherapy were performed, producing complete remission. Histology showed a predominantly follicular lymphoid infiltrate mainly composed of centroblast-like cells. The phenotype was CD20+, CD79a+, CD10+, bcl-6+, B cell specific activating protein+, κ light chain+, CD30−/+, interferon regulating factor 4−/+, c-myc−/+, λ light chain, CD3, bcl-2, p53, cytokeratin, and placental alkaline phosphatase. Lymphomatous elements were found within a CD21+ follicular dendritic cell network and 70% were positive for Ki-67/MIB-1. Molecular analysis revealed monoclonal immunoglobulin heavy chain gene rearrangement and BCL-6 mutations, in the absence of BCL-2 major breakpoint and BCL-2 minor cluster region rearrangements, p53 mutations, and death associated protein kinase gene hypermethylation.

Conclusions: These findings suggest a different pathogenesis of PPTFL compared with adult follicular lymphoma and might explain its favourable course in spite of aggressive histology.

  • follicular lymphoma
  • testis
  • childhood
  • immunohistochemistry
  • molecular biology
  • APAAP, alkaline phosphatase antialkaline phosphatase
  • BSAP, B cell specific activating protein
  • DAP, death associated protein
  • EBV, Epstein-Barr virus
  • FDC, follicular dendritic cell
  • FL, follicular lymphoma
  • Ig, immunoglobulin
  • IRF4, interferon regulating factor 4
  • ISH, in situ hybridisation
  • MGMT, 06-methylguanine-DNA-methyltransferase
  • PCR, polymerase chain reaction
  • PLAP, placental alkaline phosphatase
  • PPFLT, paediatric primary follicular lymphoma of the testis

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