Primary follicular lymphoma of the testis in childhood: an entity with peculiar clinical and molecular characteristics. Issue 9 (1st September 2002)

• Record Type: Journal Article
• Authors: 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
• Is Part Of: Journal of clinical pathology. Volume 55:Issue 9(2002)
• Page Start: 684
• Page End: 688
• Publication Date: 2002-09-01
• Subjects: 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; Pathology -- Periodicals; Pathology, Molecular -- Periodicals; 616.0705
• DOI: 10.1136/jcp.55.9.684
• Languages: English
• ISSNs: 0021-9746

Abstract

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 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 +, 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.