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

Research output: Contribution to journalArticle

43 Citations (Scopus)

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

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Follicular Lymphoma
Testis
Gene Rearrangement
Keratin-2
Histology
Death-Associated Protein Kinases
Immunoglobulin Subunits
Follicular Dendritic Cells
Pediatrics
Immunoglobulin Heavy Chain Genes
Light
Mutation
Orchiectomy
Interferons
Proteins
B-Lymphocytes
Immunohistochemistry
Phenotype
Gene Expression
Drug Therapy

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

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

In: Journal of Clinical Pathology, Vol. 55, No. 9, 09.2002, p. 684-688.

Research output: Contribution to journalArticle

Pileri, SA, Sabattini, E, Rosito, P, Zinzani, PL, 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, vol. 55, no. 9, pp. 684-688. https://doi.org/10.1136/jcp.55.9.684
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. / Primary follicular lymphoma of the testis in childhood : An entity with peculiar clinical and molecular characteristics. In: Journal of Clinical Pathology. 2002 ; Vol. 55, No. 9. pp. 684-688.
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AU - Rosito, P.

AU - Zinzani, P. L.

AU - Ascani, S.

AU - Fraternali-Orcioni, G.

AU - Gamberi, B.

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AU - Vivenza, D.

AU - Falini, B.

AU - Gaidano, G.

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