Department of Population Health Sciences, School of Public Health, Georgia State University, Atlanta, GA 30303, USA.
Department of Mathematics and Statistics, Georgia State University, Atlanta, GA 30303, USA.
Int J Environ Res Public Health. 2020 Apr 15;17(8):2710. doi: 10.3390/ijerph17082710.
Rare cancers, affecting 1 in 5 cancer patients, disproportionally contribute to cancer mortality. This research focuses on liposarcoma, an understudied rare cancer with unknown risk factors and limited treatment options. Liposarcoma incident cases were identified from the U.S. Surveillance, Epidemiology, and End Result (SEER) program and the combined SEER-National Program of Cancer Registries (CNPCR) between 2001-2016. Incidence rates (age-adjusted and age-specific), 5-year survival, and the time trends were determined using SEER*stat software. Three-dimensional visualization of age-time curves was conducted for males and females. SEER liposarcoma cases represented ~30% ( = 11,162) of the nationwide pool ( = 37,499). Both sources of data showed males accounting for ~60% of the cases; 82%-86% cases were identified among whites. Age-adjusted incidence was greater among males vs. females and whites vs. blacks, whereas survival did not differ by sex and race. The dedifferentiated (57.2%), pleomorphic (64.1%), and retroperitoneal (63.9%) tumors had the worse survival. Nationwide, liposarcoma rates increased by 19%, with the annual percent increase (APC) of 1.43% (95% confidence interval (CI): 1.12-1.74). The APC was greater for males vs. females (1.67% vs. 0.89%) and retroperitoneal vs. extremity tumors (1.94% vs. 0.58%). Thus, incidence increased faster in the high-risk subgroup (males), and for retroperitoneal tumors, the low-survival subtype. The SEER generally over-estimated the rates and time trends compared to nationwide data but under-estimated time trends for retroperitoneal tumors. The time trends suggest an interaction between genetic and non-genetic modifiable risk factors may play a role in the etiology of this malignancy. Differences between SEER and CNCPR findings emphasize the need for nationwide cancer surveillance.
罕见癌症影响了五分之一的癌症患者,不成比例地导致了癌症死亡率。本研究聚焦于脂肪肉瘤,这是一种研究不足的罕见癌症,其危险因素未知,治疗选择有限。脂肪肉瘤的发病病例是从美国监测、流行病学和最终结果(SEER)计划以及 2001-2016 年期间的 SEER-国家癌症登记处(CNPCR)联合项目中确定的。使用 SEER*stat 软件确定发病率(年龄调整和年龄特定)、5 年生存率和时间趋势。对男性和女性的年龄时间曲线进行了三维可视化。SEER 脂肪肉瘤病例占全国范围内病例的约 30%(=11162)(=37499)。两个数据源均显示男性占病例的 60%左右;82%-86%的病例在白人中发现。年龄调整发病率在男性中高于女性,在白人中高于黑人,而生存率在性别和种族之间没有差异。去分化(57.2%)、多形性(64.1%)和腹膜后(63.9%)肿瘤的生存率最差。全国范围内,脂肪肉瘤发病率增长了 19%,年增长率(APC)为 1.43%(95%置信区间(CI):1.12-1.74)。男性的 APC 高于女性(1.67%对 0.89%),腹膜后肿瘤的 APC 高于肢体肿瘤(1.94%对 0.58%)。因此,高危亚组(男性)和腹膜后肿瘤的发病率增长更快,生存率较低的亚型。与全国范围内的数据相比,SEER 通常高估了发病率和时间趋势,但低估了腹膜后肿瘤的时间趋势。这些时间趋势表明,遗传和非遗传可改变的危险因素之间的相互作用可能在这种恶性肿瘤的病因学中发挥作用。SEER 和 CNCPR 调查结果之间的差异强调了需要进行全国性癌症监测。