Kim Eun Kyung, Jang Mi, Yang Woo Ick, Yoon Sun Och
Department of Pathology, National Health Insurance Service Ilsan Hospital, Goyang 10444, Korea.
Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul 03722, Korea.
Cancers (Basel). 2021 May 29;13(11):2679. doi: 10.3390/cancers13112679.
Primary gastrointestinal T/NK cell lymphoma (GI-TNKL) is an uncommon and heterogeneous group of lymphoid malignancies. We aimed to investigate their subtype distribution, clinicopathologic characteristics, and clinical outcomes. A total of 38 GI-TNKL cases and their clinical and pathological characteristics were analyzed. GI-TNKL occurred in adults with a median patient age in the sixth decade of life and showed a slight male predominance. The most common histologic type was extranodal NK/T-cell lymphoma, nasal type (ENKTL; 34.2%), followed by monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL; 31.6%), intestinal T-cell lymphoma, NOS (ITCL, NOS, 18.4%), anaplastic large cell lymphoma, ALK-negative (ALCL, ALK-; 13.2%). The small intestine was the primary affected region. More than 90% of patients complained of various GI symptoms and cases with advanced Lugano stage, high IPI score, or bowel perforation that required emergent operation were not uncommon. GI-TNKL also showed aggressive behavior with short progression-free survival and overall survival. This thorough clinical and pathological descriptive analysis will be helpful for accurate understanding, diagnosis, and treatment.
原发性胃肠道T/NK细胞淋巴瘤（GI-TNKL）是一组罕见且异质性的淋巴恶性肿瘤。我们旨在研究其亚型分布、临床病理特征及临床结局。共分析了38例GI-TNKL病例及其临床和病理特征。GI-TNKL发生于成年人，患者年龄中位数为60岁，男性略占优势。最常见的组织学类型为结外NK/T细胞淋巴瘤，鼻型（ENKTL；34.2%），其次为单形性亲上皮性肠道T细胞淋巴瘤（MEITL；31.6%）、肠道T细胞淋巴瘤，非特指型（ITCL，NOS，18.4%）、间变性大细胞淋巴瘤，ALK阴性（ALCL，ALK-；13.2%）。小肠是主要受累部位。超过90%的患者有各种胃肠道症状，Lugano分期晚期、国际预后指数（IPI）评分高或需要紧急手术的肠穿孔病例并不少见。GI-TNKL也表现出侵袭性，无进展生存期和总生存期较短。这种全面的临床和病理描述性分析将有助于准确理解、诊断和治疗。
