Surgical Oncology Program, National Cancer Institute, Bethesda, MD, USA.
Department of Surgery, The Mount Sinai Hospital, New York, NY, USA.
Sci Rep. 2022 Apr 6;12(1):5774. doi: 10.1038/s41598-022-09745-1.
Wild-type KIT and PDGFRA gastrointestinal stromal tumors (GIST) are rare tumors with limited treatment options. We sought to determine the clinicopathologic features of wild-type GIST and identify factors that influence overall survival (OS) using a large national database. Retrospective evaluation of patients with wild-type GIST in the National Cancer Database (NCDB) was performed. Demographic, clinicopathologic, and treatment data were analyzed. Features associated with OS were investigated using Kaplan-Meier analysis and Cox proportional hazards model. 244 patients with median diagnosis age of 59 years (95% CI 57-63) were identified. The stomach was the most common primary site (57%) followed by the small intestine (35%). Surgical resection was performed on 85% of patients and 53% of patients received systemic therapy. Factors associated with decreased OS on multivariable analysis included small intestine primary (HR 2.72, 95% CI 1.13-6.69, P = 0.026) and > 5 mitoses per 50 HPF (HR 4.77, 95% CI 1.86-13.2, P = 0.001). Wild-type GISTs may be identified in older patients, with most arising in the stomach and small bowel. Surgery remains the principal treatment modality. Small intestine primary site and high mitotic count were associated with abbreviated OS.
野生型 KIT 和 PDGFRA 胃肠道间质瘤（GIST）是罕见的肿瘤，治疗选择有限。我们旨在通过大型国家数据库确定野生型 GIST 的临床病理特征，并确定影响总生存（OS）的因素。对国家癌症数据库（NCDB）中野生型 GIST 患者进行回顾性评估。分析了人口统计学、临床病理和治疗数据。使用 Kaplan-Meier 分析和 Cox 比例风险模型研究与 OS 相关的特征。确定了 244 名中位诊断年龄为 59 岁（95%CI 57-63）的患者。最常见的原发部位是胃（57%），其次是小肠（35%）。85%的患者接受了手术切除，53%的患者接受了系统治疗。多变量分析显示，OS 降低的相关因素包括小肠原发（HR 2.72，95%CI 1.13-6.69，P=0.026）和每 50HPF 超过 5 个有丝分裂（HR 4.77，95%CI 1.86-13.2，P=0.001）。野生型 GIST 可能发生在年龄较大的患者中，大多数发生在胃和小肠。手术仍然是主要的治疗方式。小肠原发部位和高有丝分裂计数与 OS 缩短相关。
