胃原发NK/T细胞淋巴瘤4例临床病理特征及文献复习

Clinicopathological characteristics of primary gastric natural killer/T-cell lymphoma and differential diagnosis:report of four cases and review of literature

目的原发胃的NK/T细胞淋巴瘤极为罕见,通过总结其临床病理特征、鉴别诊断及预后,以提高对此疾病的认识。方法回顾性分析空军军医大学第一附属医院病理科2014-01—2023-03年确诊为该疾病的病例。结果4例病例患者年龄为48~71岁(中位年龄61岁),男女比为1∶1。镜下特点以中等大小的淋巴样细胞为主,细胞核不规则、扭曲或成角,核仁明显,可见核分裂、凋亡小体及核碎屑,常可见血管侵犯,伴溃疡及坏死。免疫组化染色显示肿瘤细胞CD3(4/4)、CD56(3/4)、TIA(4/4)、Granzyme B(3/4)及CD30(3/4)阳性,Ki-67增殖指数50%~80%。4例病例原位杂交EBER均为阳性。结论该疾病比较罕见,不要漏诊及误诊。该疾病的预后需要更多的病例随访观察,但CD30的表达可为复发难治患者提供新的希望。

Objective Primary gastric natural killer/T-cell(NK/T-cell)lymphomais is extremely rare.This article summarized its clinicopathological features,differential diagnosis and prognosis to improve the understanding of this disease.Methods All the cases diagnosed as primary gastric NK/T-cell lymphoma from 2014.1-2023.3 in the First Affiliated Hospital of Air Force Military Medical University were retrospectively reviewed.Results Four patients with primary gastric NK/T-cell lymphoma ranged in age from 48 to 71 years(median age was 61 years),with a male to female ratio of 1∶1.Histologically,the tumor cells were mainly medium sized lymphoid cells with irregular,twisted or angular nuclei,obvious nucleoli,mitotic figures,apoptotic bodies and nuclear debris,and often vascular invasion,accompanied by ulceration and necrosis.Immunohistochemical staining showed positive tumor cells of CD3(4/4),CD56(3/4),TIA(4/4),Granzyme B(3/4)and CD30(3/4),Ki-67 proliferation index 50%-80%.In situ hybridization EBER was positive in all 4 cases.Conclusion Primary gastric NK/T cell lymphoma is rare and should not be missed or misdiagnosed.The prognosis of primary gastric NK/T cell lymphoma requires more follow-up observation,but the expression of CD30 can provide new hope for some patients with refractory recurrence.