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目的阐述交界性胃肠道间质瘤(gastrointestinal stromal tumors,GISTs)的特征并重新评估目前广泛应用于GISTs的美国国家卫生研究所(NIH)共识标准。方法回顾性分析840名患者的病史资料及手术切除标本。结果筛除485例恶性GISTs及76例假定的良性GISTs,剩余的279例GISTs归为交界性GISTs,其中223例随访1～31年。2例患者局部复发,随后经过单纯手术切除治愈。5年无瘤生存率和总生存率分别为99%和100%。形态学上,交界性GISTs的典型特征为富于细胞、中度异型性、低核分裂活性(<10个/50 HPF)或较大肿瘤体积。依据NIH共识标准,279例中,极低危险度组13例,低危组164例,中危组71例,高危组31例,但各组间的无瘤生存率差异无显著性(P=0.681)。结论交界性GISTs具有介于良恶性GISTs之间的临床及形态学特征。部分交界性GISTs表现出恶性潜能,需长期随访。NIH共识标准基于肿瘤体积及核分裂象划分的危险度等级并不适用于评估交界性GISTs的生物学行为。
OBJECTIVE: To characterize the borderline gastrointestinal stromal tumors (GISTs) and to reassess the NIH consensus criteria currently widely used in GISTs. Methods A retrospective analysis of 840 patients history data and surgical resection specimens. RESULTS: A total of 485 malignant GISTs and 76 presumed benign GISTs were screened out. The remaining 279 GISTs were classified as borderline GISTs, of which 223 were followed up for 1 to 31 years. Two patients with local recurrence, followed by a simple surgical excision. 5-year disease-free survival and overall survival were 99% and 100%. Morphologically, the hallmarks of borderline GISTs are cell-rich, moderately atypical, low-mitotic activity (<10 cells / 50 HPF) or larger tumor volumes. According to the NIH consensus criteria, 279 patients were classified as extremely low risk group, 13 patients in low risk group, 164 patients in low risk group, 71 in intermediate risk group and 31 in high risk group. However, there was no significant difference in tumor free survival between groups (P = 0.681). Conclusion The borderline GISTs have the clinical and morphological characteristics between benign and malignant GISTs. Some borderline GISTs show malignant potential, requiring long-term follow-up. NIH Consensus Criteria Hazard rankings based on tumor volume and mitosis are not valid for assessing the biological behavior of borderline GISTs.