HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS REGISTER		Author Keyword(s) Vol Page [Advanced]

This Article Full Text Full Text (PDF) Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Add article to my folders Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Pileri, S A Articles by Gaidano, G Search for Related Content PubMed PubMed Citation Articles by Pileri, S A Articles by Gaidano, G Pubmed/NCBI databases Medline Plus Health Information Testicular Cancer Testicular Disorders

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:
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 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⁺, 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

This article has been cited by other articles:

				Pathology Quiz Case 2 Diagnosis Arch Otolaryngol Head Neck Surg, October 1, 2007; 133(10): 1065 - 1066. [Full Text] [PDF]

				C. Aso, G. Enriquez, M. Fite, N. Toran, C. Piro, J. Piqueras, and J. Lucaya Gray-Scale and Color Doppler Sonography of Scrotal Disorders in Children: An Update RadioGraphics, September 1, 2005; 25(5): 1197 - 1214. [Abstract] [Full Text] [PDF]