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

S. A. Pileri, E. Sabattini, P. Rosito, P. L. Zinzani, S. Ascani, G. Fraternali-Orcioni, B. Gamberi, M. Piccioli, D. Vivenza, B. Falini, G. Gaidano

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Abstract

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.

Original languageEnglish
Pages (from-to)684-688
Number of pages5
JournalJournal of Clinical Pathology
Volume55
Issue number9
DOIs
Publication statusPublished - Sep 2002

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

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    Pileri, S. A., Sabattini, E., Rosito, P., Zinzani, P. L., Ascani, S., Fraternali-Orcioni, G., Gamberi, B., Piccioli, M., Vivenza, D., Falini, B., & Gaidano, G. (2002). Primary follicular lymphoma of the testis in childhood: An entity with peculiar clinical and molecular characteristics. Journal of Clinical Pathology, 55(9), 684-688. https://doi.org/10.1136/jcp.55.9.684