Department of Pathology, West China Hospital, Sichuan University, No. 37 Guo Xue Xiang, Chengdu, 610041, Sichuan, China.
Department of Dermatology, West China Hospital, Sichuan University, No. 37 Guo Xue Xiang, Chengdu, 610041, Sichuan, China.
BMC Cancer. 2018 Apr 3;18(1):373. doi: 10.1186/s12885-018-4311-z.
Epstein-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.
We 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.
To the best of our knowledge, this is the first case of EBV + PTCL with STAT5B variants of a pediatric patient presented as extranodal lesions.
EB 病毒阳性外周 T 细胞淋巴瘤（EBV+PTCL）是一种罕见的淋巴增生性疾病，通常发生在成年后期。然而，儿科 EBV+PTCL 极为罕见，通常表现为淋巴结病。此外，并非所有这些儿科患者都进行了基因检测。
我们报告了一例 9 岁儿童 EBV+PTCL，最初症状为皮下肿块，无淋巴结受累。组织学上，肿瘤细胞呈中心母细胞样，细胞核呈圆形或椭圆形，染色质略浓缩，中等大小的嗜酸性不明显核仁。免疫组化染色显示所有肿瘤细胞均阳性表达 CD8、GranzymeB 和 TIA-1。在 STAT5B 中检测到两种新型变体（S420Y 和 E623K）。
据我们所知，这是首例以结外病变为表现的儿科 EBV+PTCL 伴 STAT5B 变异的病例。
