Background: For very-rare cancers such as pheochromocytoma and paraganglioma (PPGL), center-level case volume is uniformly low, rendering the traditional volume–outcome paradigm uninformative. This study examines whether cancer programs’ institutional resources, after adjusting for tumor-specific case volume, impact overall survival (OS) after surgery.Methods: The 2004–2021 National Cancer Database was queried for patients with a diagnosis of PPGL with malignant potential. Demographics, clinicopathologic characteristics, socioeconomic status, and treatment and survival variables—together with program resource tier (high resource = Academic/Research + Comprehensive Community Cancer Programs; low resource = Community Cancer + Integrated Network Programs), were extracted. IPW-Cox proportional hazard model and survival analysis were performed.Results: 1306 patients were identified, of whom 1066 (81.6%) were treated at high-resource programs. Mean age was 59.0 years and 55.1% were female (n= 719). Median follow-up was 61.7 months (maximum 207 months). Mortality was 28.3% (n= 278). Age, race, median income, tumor size, and surgical approach did not differ by resource tier. Patients treated at high- vs. low-resource programs differed by Charlson– Deyo score (p= 0.008), gender (p= 0.033), insurance status (p= 0.004), and distance traveled to facility (p< 0.001). On adjusted survival analysis, treatment at a high-resource program was associated with improved OS (HR = 0.64,p= 0.043) and a mean survival advantage of 23 months (p= 0.009) vs. a low-resource program. Age (HR = 1.03), tumor size >10 cm (HR = 4.18), and metastasis (HR = 4.17) independently predicted worse OS.Conclusions: Despite uniformly low PPGL case volumes nationally, treatment at high-resource cancer programs was associated with a 23-month longer mean survival and a 36% lower risk of death compared with low-resource cancer programs. Further studies are needed to identify the specific institutional factors that drive this survival advantage in rare cancers.
背景:对于嗜铬细胞瘤和副神经节瘤(PPGL)这类极罕见癌症,各医疗中心的病例数量普遍偏低,使得传统的"手术量-预后"关联模式失去参考价值。本研究旨在探讨在调整肿瘤特异性病例量后,癌症诊疗机构的资源配置是否影响患者术后总生存期(OS)。 方法:从2004-2021年美国国家癌症数据库中筛选具有恶性潜能的PPGL确诊患者。提取人口统计学特征、临床病理特征、社会经济状况、治疗及生存变量,同时记录医疗机构资源层级(高资源层级=学术研究型/综合性社区癌症中心;低资源层级=社区癌症中心/综合网络诊疗机构)。采用逆概率加权Cox比例风险模型进行生存分析。 结果:共纳入1306例患者,其中1066例(81.6%)在高资源层级机构接受治疗。平均年龄59.0岁,女性占55.1%(n=719)。中位随访时间61.7个月(最长207个月)。总死亡率为28.3%(n=278)。年龄、种族、中位收入、肿瘤大小及手术方式在不同资源层级间无显著差异。高资源与低资源层级机构治疗的患者在查尔森合并症指数(p=0.008)、性别(p=0.033)、保险状态(p=0.004)及就医距离(p<0.001)方面存在差异。经校正的生存分析显示,与低资源机构相比,高资源机构治疗与OS改善相关(HR=0.64,p=0.043),平均生存获益达23个月(p=0.009)。年龄(HR=1.03)、肿瘤>10cm(HR=4.18)及转移(HR=4.17)是OS恶化的独立预测因素。 结论:尽管全国PPGL病例量普遍偏低,但高资源癌症诊疗机构的治疗相较于低资源机构,能使患者平均生存期延长23个月,死亡风险降低36%。未来需进一步研究明确驱动罕见癌症生存获益的具体机构因素。