Follicular variant of peripheral T cell lymphoma with mediastinal involvement in a child: a case report.

Abstract

Peripheral T cell lymphomas are rare in young patients. We report the first case of a follicular variant of peripheral T cell lymphoma not otherwise specified in an 11-year-old boy, who presented with a large mediastinal mass. Microscopic examination of the mediastinal biopsy revealed nodular infiltration of medium- to large-sized atypical lymphocytes. Immunohistochemistry showed expression of follicular helper T cell markers (CD10, PD1, CXCL13, and BCL6) in tumor T cells. Epstein-Barr virus (EBV) was not detected by an in situ hybridization assay for EBV-encoded RNA. Interestingly, fluorescence in situ hybridization detected the presence in the tumor cells of the t(5;9)(q33;q22) translocation, involving ITK and SYK rearrangement. T cell clonality was detected by multiplex PCR analysis of TRG and TRD gene rearrangements. After 4 cycles of systemic chemotherapy, the patient was in complete remission. Although this entity is very rare, our observations show that lymphomas arising from T follicular helper cells may occur in children and that this should be distinguished from other lymphomas, such T-lymphoblastic lymphomas, which require a specific therapeutic approach.

• Authors: Audrey Delas, Philippe Gaulard, Geneviève Plat, Pierre Brousset, Camille Laurent
• Journal: Virchows Archiv : an international journal of pathology (Virchows Arch) Vol. 466 Issue 3 Pg. 351-5 (Mar 2015) ISSN: 1432-2307 [Electronic] Germany
• PMID: 25604350 (Publication Type: Case Reports, Journal Article)

Chemical References

• Intracellular Signaling Peptides and Proteins
• Protein-Tyrosine Kinases
• SYK protein, human
• Syk Kinase
• emt protein-tyrosine kinase

Topics

• Biopsy
• Child
• Gene Rearrangement, T-Lymphocyte (genetics)
• Humans
• Intracellular Signaling Peptides and Proteins (genetics)
• Lymphoma, T-Cell, Peripheral (diagnosis, genetics, pathology)
• Male
• Mediastinal Neoplasms (diagnosis, genetics, pathology)
• Phenotype
• Protein-Tyrosine Kinases (genetics)
• Syk Kinase
• T-Lymphocytes, Helper-Inducer (pathology)