Abushalha Kamelah, Tuqan Wa'el, Albagoush Sara A, Abulaimoun Sawsan, Silberstein Peter T
Internal Medicine, Médecins Sans Frontières/Doctors Without Borders, Amman, JOR.
Department of Gastroenterology, Ochsner Health System, New Orleans, USA.
Cureus. 2020 Jun 10;12(6):e8549. doi: 10.7759/cureus.8549.
Background and objective Signet ring cell carcinoma of the appendix (SRCCA) is an exceedingly rare tumor, and very limited data are available regarding its characteristics and survival probabilities. Our objective in this study was to utilize the Surveillance, Epidemiology, and End Results (SEER) database to explore the patient and tumor characteristics and to characterize the three- and five-year cancer-specific survival (CSS) probabilities of SRCCA. Methods Patients with SRCCA diagnosed between 2000 and 2015 were analyzed using the SEER database. The three- and five-year CSS probabilities were estimated by the Kaplan-Meier method, and the groups were compared using log-rank comparisons and multivariable Cox hazard regression analysis. Results A total of 527 patients were identified. The median age of the participants at diagnosis was 56 years, with a majority of them being female and white. Histologically, 60% of the tumors were high grade, and 61.3 % of the tumors were found to be metastatic on presentation. Three- and five-year CSS probabilities were 39% and 18.4%, respectively, and median survival was 26 months. Best survival outcomes were noted in males (five-year CSS: 25.4%, p=0.027), unmarried patients (five-year CSS: 19.1%, p=0.042), tumors <2 cm in size (five-year CSS: 50.5%, p<0.001), and low-grade tumors (five-year CSS: 44.8%, p<0.001). Subtotal colectomy yielded better three- and five-year CSS probabilities compared to no surgery and partial colectomy (48.5% and 26.5%, respectively, p<0.001). On the multivariate analysis, it was found that age and stages T4, N1, and M1 were associated with an increased risk of mortality, while surgery, regardless of the extent, was a protective factor. Conclusion SRCCA is a rare tumor with a high prevalence among old-aged white females. This tumor is usually diagnosed in an advanced stage and has a dismal prognosis. Surgical intervention, regardless of the extent, showed better survival probabilities compared to no surgery.
背景与目的 阑尾印戒细胞癌（SRCCA）是一种极其罕见的肿瘤，关于其特征和生存概率的数据非常有限。本研究的目的是利用监测、流行病学和最终结果（SEER）数据库，探讨患者和肿瘤特征，并描述SRCCA的3年和5年癌症特异性生存率（CSS）。方法 使用SEER数据库分析2000年至2015年间诊断为SRCCA的患者。采用Kaplan-Meier法估计3年和5年CSS概率，并使用对数秩检验和多变量Cox风险回归分析对各亚组进行比较。结果 共识别出527例患者。参与者诊断时的中位年龄为56岁，其中大多数为女性和白人。组织学上，60%的肿瘤为高级别，61.3%的肿瘤在初诊时已发生转移。3年和5年CSS概率分别为39%和18.4%，中位生存期为26个月。男性（5年CSS：25.4%，p=0.027）、未婚患者（5年CSS：19.1%，p=0.042）、肿瘤大小<2 cm（5年CSS：50.5%，p<0.001）和低级别肿瘤（5年CSS：44.8%，p<0.001）的生存结局最佳。与未手术和部分结肠切除术相比，次全结肠切除术的3年和5年CSS概率更高（分别为48.5%和26.5%，p<0.001）。多变量分析发现，年龄以及T4、N1和M1期与死亡风险增加相关，而手术无论范围如何，都是一个保护因素。结论 SRCCA是一种罕见肿瘤，在老年白人女性中患病率较高。这种肿瘤通常在晚期被诊断出来，预后较差。与未手术相比，无论手术范围如何，手术干预的生存概率更高。
