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Extranodal Natural Killer/T-Cell Lymphoma in Children and Adolescents:  A Report of 17 Cases in China.

中国儿童和青少年结外NK/T细胞淋巴瘤:附17例病例报告

Huang Y,Xie J,Ding Y,Zhou X

Abstract

To investigate the clinicopathologic features of extranodal natural killer/T-cell lymphoma (ENKTL) not preceded by another Epstein-Barr virus (EBV)-related disorder in children and adolescents.
A series of 17 cases of ENKTL not preceded by another EBV-related disorder in children and adolescents in one institution from China was retrospectively analyzed.
The patients had a median age of 13 years (range, 2-17 years), and the male to female ratio was 3.25:1. All the patients were of Chinese origin and without a history of another EBV-related disorder. The upper aerodigestive tract was involved in 14 (82.4%) cases, of which the sinonasal region was involved in 10 (58.8%) cases. Twelve (70.6%) patients had stage I/II disease. Morphologically, coagulative necrosis and angiocentric/angiodestructive growth pattern were observed in 94.1% (16/17) and 88.2% (15/17) of all cases, respectively. Thirteen (76.5%) cases were mainly composed of medium-sized cells. All cases expressed cytoplasmic CD3 and at least one marker for cytotoxic granules. CD56 expression was seen in 16 (94.1%) of 17 cases. Each case harbored EBV.
ENKTL not preceded by another EBV-related disorder in children and adolescents is occasionally observed in China. It tends to have a male predominance. The upper aerodigestive tract, especially the sinonasal region, is the most commonly involved site. The morphologic features and immunophenotype in children and adolescents are similar to that occurring in adults.

摘要

探讨既往无EBV相关疾病的儿童和青少年结外NK/T细胞淋巴瘤临床病理特征。

回顾性分析我院17儿童和青少年结外NK/T细胞淋巴瘤资料。

患者中位年龄为13岁(2-17岁),男女比例为3.25:1。所有患者为中国国籍,既往无EBV相关疾病。累及上消化道14例(82.4%)其中鼻腔鼻窦区10 例(58.8%)。12例(70.6%)临床分期为I或II期。组织形态学上,凝固性坏死和血管中心性/血管破坏性生长模式分别出现于94.1% (16/17) 和 88.2% (15/17)病例中。13例(76.5%)主要由中等大小细胞组成。所有病例均表达胞质型CD3和至少一种细胞毒性颗粒标记。CD56在94.1%(16/17)病例中表达,所有病例EBV均阳性。

既往无EBV相关疾病的中国儿童和青少年结外NK/T细胞淋巴瘤比较少见,患者以男性为主。上消化道和鼻腔鼻窦区是最常见的累及部位。发生于儿童和青少年的结外NK/T细胞淋巴瘤形态学和免疫表型与成人相似。

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