肠套叠为首发表现儿童原发性胃肠道淋巴瘤临床特征分析

Clinical cases analysis of primary gastrointestinal lymphoma with intussusception as the first presentation

目的回顾性分析儿童原发性胃肠道淋巴瘤(primary gastrointestinal lymphoma,PGIL)合并肠套叠的临床资料并总结其特点。方法选取南京医科大学附属南京儿童医院2009-01-01-2014-12-31入院诊断为"肠套叠",且出院诊断包含"淋巴瘤"的患者为研究对象。参照Dawson标准筛选出PGIL患者,排除术中确诊为胃肠道肿块而并非肠套叠的患者,对同一患者多次入院者,收集其全部资料并进行回顾性分析。肿瘤分期按St.Jude标准,对所有患者进行随访,包括其化疗情况、化疗后评估结果及目前生活状况。结果共有13例PGIL患者以肠套叠为首发表现,年龄1.3~11岁,年龄>2岁12例,其中7例症状反复出现。13患者例均经过手术治疗,其中10例术中均发现套头部有异常包块存在,1例横结肠套叠肠壁增厚、僵硬和坏死,1例多发性小肠息肉状隆起,1例因套叠无法复位而切除回盲部。13例患者均成功行局部病灶切除术,术后恢复良好,未发现有吻合口瘘和吻合口狭窄等并发症者。术后病理Burkitt淋巴瘤7例,弥漫性大B细胞淋巴瘤5例,淋巴母细胞淋巴瘤(B细胞性)1例。2例肿瘤分期Ⅲ期,其余11为例Ⅱ期。除患者3未化疗外,其余患者均在病理明确诊断后行规范化疗。随访时间10~69个月,中位随访时间39个月,除患者3在术后11个月死亡外,其余患者未发现有肿瘤复发者,无病生存率(event free survival,EFS)为92.3%。结论对于非典型发病年龄或反复发作的肠套叠,应注意由PGIL引起的继发性肠套叠,手术中如果发现肠壁包块应切除病理检查,确诊为PGIL后及时化疗,预后多良好。

OBJECTIVE Primary gastrointestinal lymphoma complicated with intussusception is not very rare, but early diagnosis is rather difficult. To summarize clinical features of this secondary intussusception, clinical data in our hos pital were analyzed retrospectively. METHODS From 2009.1 to 2014.12, patients records in Nanjing children＇s Hospital were retrieved for cases whose initial diagnosis was intussusception and discharge diagnosis included search field of lyre phoma. Dawson＇s Criteria was used to screen out PGIL cases. Those cases were excluded if proved intraoperatively to be intestinal mass rather than intussusception. For patients with frequent hospital readmissions, record data and material was collected and analyzed retrospectively. The St Jude （Murphy） staging system was used to determine disease stage. Patients were followed up to investigate their quality of life, executive condition and effect of chemotherapy. RESULTS During the last 6 years, 13 patients with PGIL presented as intussusception initially, age from 1.3 to 11 （12 cases〉2 years old）, while intussusception appeared repeatedly in 7 cases. All 13 cases received surgical treatment, among them, abnormal lumps were found at intussusceptum in 10 cases, local colic wall thickened and necrosis in 1 case, multiple intestinal poly poid prominences in 1 case. Local resection was performed successfully for each case without postoperative complication of anastomotic leakage or stricture. Pathological examination revealed 7 cases of Burkitt^s lymphoma, 5 of diffuse large B cell lymphoma and 1 of Bcell lymphoblastic lymphoma. 2 cases were classified to be tumor stage I]I, the remaining 11 cases to be stage II. 12 cases were transferred into oncology department and treated with chemotherapy, except case 3 who died 11 months after operation. During a median follow-up period of 39 months （ranged from 10 to 69 months）, tumor recur- rence had not been found in these 12 case （Event Free Survival rate-92.3%）. CONCLUSIONS For children with recurrent intussusception or episode at atypical age, PGIL should be concerned. Abnormal mass on intestinal wall should be resected for Pathological examination. Early postoperative chemotherapy can provide children with PGIL a good prognosis.