Abstract
BackgroundEpstein-Barr virus positive peripheral T cell lymphoma (EBV + PTCL) is a rare type of lymphoproliferative disorder which is always present in late adulthood. However, pediatric EBV + PTCL is extremely rare and always present with lymphadenopathy. Additionally, gene detection was not performed in all of these pediatric patients.Case presentationWe report an EBV + PTCL in a 9-year-old child with initial symptom of subcutaneous masses without lymph node involvement. Histologically, the neoplastic cells were centroblastoid with round or oval nuclei, slightly condensed chromatin and median eosinophilic inconspicuous nucleoli. Immunohistochemically, all neoplastic cells were positive for CD8, GranzymeB and TIA-1. Two novel variants (S420Y and E623K) were detected in STAT5B.ConclusionTo the best of our knowledge, this is the first case of EBV + PTCL with STAT5B variants of a pediatric patient presented as extranodal lesions.
Highlights
Epstein-Barr virus positive peripheral T cell lymphoma (EBV + PTCL) is a rare type of lymphoproliferative disorder which is always present in late adulthood
EBV- associated T/ NK lymphoproliferative disorder occupied a large part in Asian pediatric patients [3], EBV + PTCL is extremely rare, with only 4 patients of childhood have been reported [4, 5]
EBV + PTCL, as a variant of PTCL-NOS, is a rare malignancy which is more frequent in the late adulthood with high clinical stage and aggressive clinical course [2]. for the patients of childhood, it was extremely rare with limited cases have been reported (Additional file 4: Table S1) [4, 5, 7]
Summary
Peripheral T cell lymphoma, not otherwise specific (PTCL-NOS) is the most common type of T cell lymphoma in adulthood which is account for 25.9% in Western countries [1]. EBV- associated T/ NK lymphoproliferative disorder occupied a large part in Asian pediatric patients [3], EBV + PTCL is extremely rare, with only 4 patients (including this case) of childhood have been reported [4, 5] Among these patients, the primary sites were lymph nodes with or without extranodal involvement, and the genetic detection. Medical history A 9-year-old boy was admitted to pediatric department with chief complaining of multiple subcutaneous masses for 2 months During this period, the patient had no fever, lymphadenopathy or hepatosplenomegaly. Treatment and follow up After diagnosis, a positron emission tomography–computed tomography (PET-CT) was applied and revealed an increasing 2-Deoxy-2-fluoro-D-glucose (18F-FDG) uptake of multiple masses in subcutaneous tissue and muscles but no lymph node was involved (Additional file 3: Figure S2A). 3 months after the treatment, another subcutaneous mass was palpable in the right thigh and the excisional biopsy suggested relapse (Fig. 1e, f, g and h)
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