Zhou Lei, Liao Yusheng, Wu Jie, Yang Jing, Zhang Heng, Wang Xiangyang, Sun Shengbin
Department of Gastroenterology, The Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, Peoples' Republic of China,
Key Laboratory for Molecular Diagnosis of Hubei Province, The Central Hospital of Wuhan, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Peoples' Republic of China.
Ther Clin Risk Manag. 2018 Aug 22;14:1467-1481. doi: 10.2147/TCRM.S167248. eCollection 2018.
Gastrointestinal stromal tumor (GIST) is a rare tumor of the small bowel, which can be difficult to diagnose and has a varied clinical outcome.
This is a retrospective review of the diagnosis, management, and clinical outcome of 32 patients diagnosed with primary small bowel GIST from a single center and a comparison of the findings with previously published cases.
Retrospective review of data from patient clinical records, endoscopic and imaging findings, surgical procedures, tumor histology and immunohistochemistry, and clinical outcome was conducted.
Data of 32 patients with a median age of 56 years including 50% men and women were reviewed. The majority (29/32) were symptomatic at presentation, with the main symptom being gastrointestinal bleeding (15/32). Imaging detection rates included ultrasound (0%), magnetic resonance imaging (0%), computed tomography (54.8%), computed tomography angiography (71.4%), and double-balloon enteroscopy (88.9%). The mean tumor diameter was 5.3 cm; 4 tumors were located in the duodenum, 21 in the jejunum, and 7 in the ileum. Based on the tumor size and mitotic index, 5 (15.6%), 15 (46.9%), 0 (0%), and 12 (37.5%) patients were classified into very low-risk, low-risk, intermediate-risk, and high-risk groups. Immunohistochemistry showed positive expression for CD117 (100%), CD34 (81.2%), DOG1 (93.8%), smooth muscle actin (37.5%), S100 (9.4%), and desmin (6.2%). Twenty-five patients (78.1%) were treated with open surgical tumor resection; seven patients (21.9%) underwent laparoscopic surgery. Postoperative complications that occurred in seven patients (21.9%) were resolved with conservative management. Four patients were treated with postoperative imatinib. At median follow-up of 30 months, two patients were died.
The findings from this case series, combined with the findings from previously published cases, provide an update on the current status of the diagnosis and the therapeutic approaches that might lead to improvement in prognosis for patients who present with primary small bowel GIST.
胃肠道间质瘤(GIST)是一种罕见的小肠肿瘤,诊断困难且临床结局各异。
对来自单一中心的32例原发性小肠GIST患者的诊断、治疗及临床结局进行回顾性分析,并将结果与既往发表的病例进行比较。
对患者临床记录、内镜及影像学检查结果、手术过程、肿瘤组织学及免疫组化结果以及临床结局的数据进行回顾性分析。
共纳入32例患者,中位年龄56岁,男女各占50%。大多数患者(29/32)就诊时有症状,主要症状为胃肠道出血(15/32)。影像学检查的检出率分别为:超声(0%)、磁共振成像(0%)、计算机断层扫描(54.8%)、计算机断层扫描血管造影(71.4%)和双气囊小肠镜检查(88.9%)。肿瘤平均直径为5.3 cm;4例位于十二指肠,21例位于空肠,7例位于回肠。根据肿瘤大小和有丝分裂指数,5例(15.6%)、15例(46.9%)、0例(0%)和12例(37.5%)患者分别被分为极低风险、低风险、中风险和高风险组。免疫组化显示CD117阳性表达率为100%,CD34为81.2%,DOG1为93.8%,平滑肌肌动蛋白为37.5%,S100为9.4%,结蛋白为6.2%。25例患者(78.1%)接受了开放性手术肿瘤切除;7例患者(21.9%)接受了腹腔镜手术。7例患者(21.9%)发生的术后并发症经保守治疗后得到缓解。4例患者术后接受了伊马替尼治疗。中位随访3个月时,2例患者死亡。
本病例系列的结果,结合既往发表病例的结果,为原发性小肠GIST患者的诊断现状及可能改善预后的治疗方法提供了最新信息。