Zhou Jun, Yu Bo, He Yan, Wu Bo, Zhang Xin-hua, Zhou Xiao-jun, Shi Qun-li
Department of Pathology, Clinical School of Nanjing University Medical College, Nanjing General Hospital of PLA, Nanjing 210002, China.
Zhonghua Bing Li Xue Za Zhi. 2013 Apr;42(4):243-7. doi: 10.3760/cma.j.issn.0529-5807.2013.04.007.
To study the clinicopathologic features, diagnosis and differential diagnosis of intestinal natural killer (NK)/T-cell lymphoma.
The clinical features, histopathology, immunohistochemical findings and follow-up data of 14 cases of intestinal NK/T-cell lymphoma were retrospectively reviewed.
The male-to-female ratio was 9:5. The medium age of patients was 45 years. The sites of involvement included small intestine (6 cases), colon (6 cases) or both (2 cases). The main clinical manifestations were an abdominal mass, other gastrointestinal symptoms such as abdominal pain, as well as systemic symptoms such as fever and cachexia. Intestinal perforation complicated by acute peritonitis might occur in advanced disease. Histologically, the intestinal wall showed full-thickness infiltration by medium-sized atypical lymphoid cells with pleomorphic nuclei, prominent inflammatory background, angiocentric/angiodestructive growth pattern and coagulative necrosis. Immunohistochemical study showed that the tumor cells were positive for CD3ε, CD43, CD56, granzyme B and perforin. They were negative for CD20, CD79α and MPO. In-situ hybridization for Epstein-Barr virus encoded RNA (EBER) showed negative signals. A high proliferative index was demonstrated by Ki-67 immunostaining. Follow-up data of 8 cases were available, with duration of follow up ranging from 0.5 to 36 months. Five patients died within 20 months.
Extranodal NK/T-cell lymphoma, nasal-type primarily involving intestine is rare and tends to carry an aggressive clinical course. The relatively non-specific clinical manifestations of intestinal NK/T-cell lymphoma may result in misdiagnosis in some cases. A comprehensive evaluation of clinical manifestations, pathologic features and immunohistochemical findings is essential for definitive diagnosis.
研究肠道自然杀伤（NK）/T细胞淋巴瘤的临床病理特征、诊断及鉴别诊断。
回顾性分析14例肠道NK/T细胞淋巴瘤的临床特征、组织病理学、免疫组化结果及随访资料。
男女比例为9:5。患者中位年龄为45岁。受累部位包括小肠（6例）、结肠（6例）或两者均受累（2例）。主要临床表现为腹部肿块、腹痛等其他胃肠道症状以及发热、恶病质等全身症状。晚期疾病可能发生肠穿孔并发急性腹膜炎。组织学上，肠壁显示为中等大小的非典型淋巴细胞全层浸润，细胞核多形性，炎症背景突出，血管中心性/血管破坏性生长模式及凝固性坏死。免疫组化研究显示肿瘤细胞CD3ε、CD43、CD56、颗粒酶B和穿孔素呈阳性。CD20、CD79α和MPO呈阴性。爱泼斯坦-巴尔病毒编码RNA（EBER）原位杂交显示阴性信号。Ki-67免疫染色显示增殖指数高。8例有随访资料，随访时间为0.5至36个月。5例患者在20个月内死亡。
结外NK/T细胞淋巴瘤，鼻型，主要累及肠道者罕见，临床病程往往呈侵袭性。肠道NK/T细胞淋巴瘤相对非特异性的临床表现可能导致某些病例误诊。对临床表现、病理特征和免疫组化结果进行综合评估对于明确诊断至关重要。
