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

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 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(+), kappa light chain(+), CD30(-/+), interferon regulating factor 4(-/+), c-myc(-/+), lambda 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.

• Authors: 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
• Journal: Journal of clinical pathology (J Clin Pathol) Vol. 55 Issue 9 Pg. 684-8 (Sep 2002) ISSN: 0021-9746 [Print] England
• PMID: 12194999 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• DNA-Binding Proteins
• Proto-Oncogene Proteins
• Proto-Oncogene Proteins c-bcl-6
• Transcription Factors

Topics

• Child, Preschool
• DNA-Binding Proteins (genetics)
• Gene Rearrangement, B-Lymphocyte, Heavy Chain
• Humans
• Immunophenotyping
• Lymphoma, Follicular (genetics, immunology, pathology)
• Male
• Proto-Oncogene Proteins (genetics)
• Proto-Oncogene Proteins c-bcl-6
• Testicular Neoplasms (genetics, immunology, pathology)
• Transcription Factors (genetics)