ORIGINAL ARTICLE

Primary follicular lymphoma of the testis in childhood: an entity with peculiar clinical and molecular characteristics

S A Pileri¹, E Sabattini¹, P Rosito², P L Zinzani¹, S Ascani¹, G Fraternali-Orcioni¹, B Gamberi¹, M Piccioli¹, D Vivenza³, B Falini⁴ and G Gaidano³

¹ Pathology and Clinical Units of the Institute of Haematology and Clinical Oncology "L. and A. Seràgnoli", University of Bologna, 40138 Bologna, Italy
² Unit of Paediatric Haematology and Oncology, Department of Paediatrics, University of Bologna
³ Division of Internal Medicine, Department of Medical Sciences, University of Eastern Piedmont "Amedeo Avogadro", 28100 Novara, Italy
⁴ Haematopathology Laboratory, Institute of Haematology, University of Perugia, 06100 Perugia, Italy

Correspondence to:
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;
pileri{at}almadns.unibo.it

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 V_H chain rearrangements.

Results: The neoplasm was staged I^E/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⁺, light chain⁺, CD30^-/+, interferon regulating factor 4^-/+, c-myc^-/+, 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.

Keywords: follicular lymphoma; testis; childhood; immunohistochemistry; molecular biology

Abbreviations: 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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