Department of Pathology, The First Affiliated Hospital of Zhengzhou University, 1 Jianshe Road, Zhengzhou, 450052, Henan, People's Republic of China.
Department of Oncology, The First Affiliated Hospital of Zhengzhou University, 1 Jianshe Road, Zhengzhou, 450052, Henan, People's Republic of China.
Sci Rep. 2022 May 3;12(1):7118. doi: 10.1038/s41598-022-11247-z.
Natural killer/T-cell lymphoma (NKTCL) in children and adolescents is a rare type of T/NK cell neoplasms. The aim of the present study was to analyze the clinicopathological and genetic features of this rare entity of lymphoma. We evaluated the clinical, histopathological and molecular features of 22 young people with NKTCL, including 15 males and 7 females, with a median age of 15 years. The results revealed that the nasal site was the most involved region while non-nasal sites were observed in 27.3% out of all cases. The tumor cells were composed of small‑sized to large cells and 19 (86.4%) cases exhibited coagulative necrosis. The neoplastic cells in all patients were positive for CD3 and the cytotoxic markers. Nineteen (86.4%) cases were positive for CD56. Reduced expression of CD5 was observed in all available cases. CD30 was heterogeneously expressed in 15 (75.0%) cases. All 22 patients were EBV positive. Seven (36.8%) out of all the 19 patients during the follow-up died of the disease, and the median follow‑up period was 44 months. Moreover, patients treated with radiotherapy/chemotherapy showed significantly inferior OS compared with the untreated patients. High mutation frequencies were detected including KMT2C (5/5), MST1 (5/5), HLA-A (3/5) and BCL11A (3/5), which involved in modifications, tumor suppression and immune surveillance. These results suggest that NKTCL in children and adolescents exhibits histopathological and immunohistochemical features similar to the cases in adults. Active treatment is necessary after the diagnosis of NKTCL is confirmed. Furthermore, genetic analyse may provide a deep understanding of this rare disease.
儿童和青少年自然杀伤细胞/T 细胞淋巴瘤（NKTCL）是一种罕见的 T/NK 细胞肿瘤。本研究旨在分析这种罕见淋巴瘤实体的临床病理和遗传特征。我们评估了 22 例 NKTCL 年轻人的临床、组织病理学和分子特征，包括 15 例男性和 7 例女性，中位年龄为 15 岁。结果显示，鼻腔是最常见的受累部位，而所有病例中有 27.3%存在非鼻腔部位受累。肿瘤细胞由小至大细胞组成，19 例（86.4%）存在凝固性坏死。所有患者的肿瘤细胞均表达 CD3 和细胞毒性标志物。19 例（86.4%）患者表达 CD56。所有可评估病例均表现为 CD5 表达减少。CD30 在 15 例（75.0%）患者中呈异质性表达。所有 22 例患者均 EBV 阳性。19 例患者中有 7 例（36.8%）在随访期间死于该疾病，中位随访时间为 44 个月。此外，接受放疗/化疗的患者的总生存期明显劣于未治疗的患者。检测到高突变频率，包括 KMT2C（5/5）、MST1（5/5）、HLA-A（3/5）和 BCL11A（3/5），这些基因涉及修饰、肿瘤抑制和免疫监视。这些结果表明，儿童和青少年 NKTCL 具有与成人相似的组织病理学和免疫组织化学特征。确诊 NKTCL 后需要积极治疗。此外，遗传分析可能为深入了解这种罕见疾病提供帮助。
