© 2002 Journal of Clinical Pathology
ORIGINAL ARTICLE
Primary follicular lymphoma of the testis in childhood: an entity with peculiar clinical and molecular characteristics
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 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+,
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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