Background:Site-specific long-term outcomes, including neurofibromatosis type 1 (NF1), Ki-67 prognostic value, and very late recurrences of small bowel gastrointestinal stromal tumors (GISTs), remain inadequately defined.Methods:This retrospective cohort study investigated the clinical characteristics, diagnostic challenges, and long-term outcomes of patients with small bowel GISTs. This retrospective, single-center study (2008–2024) analyzed 27 consecutive patients (average age: 62.2 years) with jejunal/ileal GISTs. Clinicopathologic features, diagnostic yield of balloon-assisted enteroscopy (BAE), treatments, and outcomes were evaluated during a 10.2-year median follow-up period. Recurrence-free survival (RFS) and overall survival (OS) were estimated by Kaplan–Meier with log-rank testing. Ki-67 was assessed using MIB-1; a prespecified 5% cut-off was chosen based on prior evidence.Results:Tumor (mean size, 62.4 mm) sites included the jejunum (74.1%) and ileum (25.9%). NF1 was present in 3/27 (11.1%) patients, all with multiple jejunal tumors. Among the 14 patients who underwent BAE, biopsy was attempted in six and yielded a histological diagnosis in one (16.7%). Six patients had recurrence; two died from disease >10 years postoperatively. Five-year OS and RFS were 91.3% and 68.7%, respectively. Adverse RFS was associated with ileal location (p= 0.03), size ≥ 10 cm (p< 0.001), mitoses > 5/50 high-power fields (p= 0.002), and Ki-67 ≥ 5% (p< 0.001). One patient labeled low risk by conventional models had recurrence with Ki-67 = 10%. Another classified as low risk by conventional models experienced recurrence >10 years after surgery, with a Ki-67 index of 10%.Conclusions:Extended, risk-adapted surveillance may be reasonable for small-bowel GISTs, and it may be beneficial to incorporate Ki-67 (≥5%) into site-based risk stratification. These observations remain hypothesis-generating and require validation in larger, multicenter cohorts and prospective studies.
背景：特定部位胃肠道间质瘤（GISTs）的长期预后情况，包括1型神经纤维瘤病（NF1）、Ki-67的预后价值以及小肠GISTs的极晚期复发，目前仍未明确。方法：本回顾性队列研究旨在探讨小肠GISTs患者的临床特征、诊断挑战及长期预后。这项回顾性单中心研究（2008–2024年）连续纳入了27例空肠/回肠GISTs患者（平均年龄：62.2岁）。在中位随访10.2年期间，评估了患者的临床病理特征、球囊辅助小肠镜（BAE）的诊断率、治疗方案及结局。采用Kaplan-Meier法和log-rank检验估算无复发生存期（RFS）和总生存期（OS）。使用MIB-1抗体评估Ki-67；基于先前证据，预设5%作为截断值。结果：肿瘤平均大小为62.4 mm，部位包括空肠（74.1%）和回肠（25.9%）。3/27例（11.1%）患者伴有NF1，且均患有多发性空肠肿瘤。在14例接受BAE检查的患者中，6例尝试了活检，其中1例（16.7%）获得了组织学诊断。6例患者出现复发；2例在术后超过10年死于本病。五年OS和RFS分别为91.3%和68.7%。RFS不良与回肠部位（p=0.03）、肿瘤尺寸≥10 cm（p<0.001）、核分裂象>5/50高倍视野（p=0.002）及Ki-67≥5%（p<0.001）相关。1例被传统模型归为低风险的患者（Ki-67=10%）出现了复发。另1例被传统模型归为低风险的患者在术后超过10年复发，其Ki-67指数为10%。结论：针对小肠GISTs，延长且基于风险分层的监测可能是合理的，将Ki-67（≥5%）纳入基于部位的风险分层可能有益。这些发现仍需视为假设，需要在更大规模的多中心队列和前瞻性研究中加以验证。
肿瘤(癌症)患者之家