肠原性结外鼻型NK/T细胞淋巴瘤1例MDT讨论

MDT discussion of a case of primary intestinal extranodal nasal type NK/T-cell lymphoma

目的总结肠原性结外鼻型NK/T细胞淋巴瘤(primary intestinal extranodal nasal type NK/T-cell lymphoma,PI-ENKTCL)的诊疗及临床病理特点,提高对此罕见病的认识。方法回顾性分析甘肃省人民医院于2019年1月诊治的1例PI-ENKTCL患者的临床资料。结果该患者行PET-CT检查提示可疑PI-ENKTCL,经MDT讨论后决定手术切除病灶,且成功行R0切除。手术历时280 min,术中出血约50 mL,术后病理回报切缘阴性,病理类型符合PI-ENKTCL诊断。术后无明显手术并发症,于术后13 d出院。术后已获访5个月,规律行吉西他滨+奥沙利铂联合左旋门冬酰胺酶(L-GMOEX)化疗方案4期,未见复发和转移,继续随访治疗效果。结论PI-ENKTCL病例罕见,可疑该诊断时须经MDT讨论。影像学检查以PET-CT检查为主。治疗以手术切除为基础,术后根据病理结果制定最佳化疗方案,以改善患者预后及延长患者生存时间。

Objective To summarize the diagnosis and clinicopathological features of primary intestinal extranodal nasal type NK/T-cell lymphoma(PI-ENKTCL), and to enhance the diagnostic and differential diagnosis of this rare disease. Methods The clinicopathologic data of a patient with PI-ENKTCL admitted to Gansu Provincial Hospital in January 2019 were retrospectively analyzed. Results We diagnosed PI-ENKTCL doubtfully by PET-CT,and after the discussion of MDT, we finished radical operation which achieved R0 resection. The pathological type was consistent with PI-ENKTCL diagnosis. The operation lasted 280 minutes, and the intraoperative blood loss was about 50 mL.Postoperative pathology confirmed that the tumor margin was negative and no postoperative complication occurred, and the patient was discharged successfully on the 13th day after the operation. The patient, followed for five months after operation, was treated with L-GMOEX [gemcitabine+oxaliplatin combined with L-asparaginase(L-ASP)] chemotherapy for four periods at time, and the treatment effect continued to be followed up. Conclusions It is necessary to discuss by MDT for doubtfully diagnosing PI-ENKTCL because of the disease being rare. The imaging diagnosis is mainly PETCT. Surgical resection is the main treatment, we should formulate the best chemotherapy according to post-operation pathological results, for improving the patient’s prognosis and prolonging patient’s life with PI-ENKTCL.