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

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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.

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Author contributions

AD, CL, and PB made the diagnosis and wrote the report. PG performed the ITK/SYK FISH study. PB, PG and GP revised the report.

Conflict of interest

We declare that we have no conflicts of interest.

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Authors and Affiliations

  1. Laboratoire d’Anatomie Pathologique Institut Universitaire du Cancer Toulouse Oncopole, 31100, Toulouse, France

    Audrey Delas, Pierre Brousset & Camille Laurent

  2. Department of Pathology, AP-HP, Groupe Hospitalier Henri Mondor—Albert Chenevier, Créteil, France

    Philippe Gaulard

  3. Université Paris-Est, Faculté de Médecine, Créteil, France

    Philippe Gaulard

  4. INSERM, U955, Institut Mondor de Recherche Biomédicale, Créteil, France

    Philippe Gaulard & Camille Laurent

  5. Service d’Oncologie Pédiatrique CHU Purpan, 31300, Toulouse, France

    Geneviève Plat

  6. INSERM, U.1037, Centre de Physiopathologie de Toulouse-Purpan, 31100, Toulouse, France

    Pierre Brousset & Camille Laurent

Authors
  1. Audrey Delas
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  2. Philippe Gaulard
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  3. Geneviève Plat
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  4. Pierre Brousset
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  5. Camille Laurent
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Correspondence to Camille Laurent.

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Delas, A., Gaulard, P., Plat, G. et al. Follicular variant of peripheral T cell lymphoma with mediastinal involvement in a child: a case report. Virchows Arch 466, 351–355 (2015). https://doi.org/10.1007/s00428-015-1716-9

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  • DOI: https://doi.org/10.1007/s00428-015-1716-9

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