Onyemkpa Chibueze, Davis Alan, McLeod Michael, Oyasiji Tolutope
Department of Surgery, Michigan State University, Lansing, MI, USA.
Barbara Ann Karmanos Cancer Institute at McLaren Flint, Department of Oncology, Wayne State University, Detroit, MI, USA.
J Gastrointest Oncol. 2019 Apr;10(2):300-306. doi: 10.21037/jgo.2018.11.08.
Goblet cell carcinoids (GCC) and other atypical neuroendocrine tumors (NET) of the appendix as well as appendiceal adenocarcinoma are usually managed with the same algorithm as colon cancers. This study investigates clinicopathological features that are predictive of survival outcomes for appendiceal NET and adenocarcinoma. Survival profiles for the histologic subtypes of appendiceal NET and adenocarcinoma were compared.
A retrospective review of appendiceal NET and adenocarcinoma for patients who are 18 years and above in the SEER database from 2010 to 2014.
Females outnumbered males in a 1.3 to 1 ratio in the NET subgroup and 1.1 to 1 ratio in the adenocarcinoma group. The mean age at diagnosis for all NET was 50.3±17 years while that of adenocarcinomas was 60.8±14.1 years. Within the NET subgroups, the mean ages for typical carcinoids (TC), GCC, NEC and MANEC were 42.9±17.3, 56.7±13.7, 45.6±17.4 and 59.7±12.8 years, respectively. Overall survival for adenocarcinoma was 86.3%, 73.5%, 65.7%, and 57.6% for 1-, 2-, 3- and 4-year OS, respectively. For NET, TC showed better survival profile with 1- and 4-year overall survival of 97.4% and 95.7%, respectively while MANEC had the worst survival outcome with 1- and 4-year OS of 88.6% and 62.2%, respectively. GCC had a better 1-year OS compared to NEC (95.5% versus 92.9%) but showed slightly worse 4-year OS (82% versus 84.8%). Age at diagnosis (HR 1.03), African-American race (HR 1.47) and stage IV disease (HR 9.58) were independent predictors of survival for appendiceal adenocarcinoma. For NET, advanced age at diagnosis, advanced disease stage and the African-American race were identified as negative independent predictors of survival.
While prior studies have suggested that atypical NET (GCC, NEC and MANEC) are more likely to present at more advanced stages, this study showed that most cases of GCC, MANEC and NEC were diagnosed at stages I and II. Appendiceal adenocarcinoma, on the other hand, presented mostly at stage IV. With respect to OS, atypical histologic subtypes of NET have worse outcome compared to TC. Although better OS was noted for GCC, NEC and MANEC when compared to adenocarcinoma, this benefit was lost in stage IV disease where adenocarcinoma recorded better 1- and 4-year OS. Prospective and randomized studies which provide granular details of treatment are needed to better define treatment algorithm for appendiceal NET.
阑尾杯状细胞类癌（GCC）及其他非典型神经内分泌肿瘤（NET）以及阑尾腺癌的治疗方案通常与结肠癌相同。本研究调查了可预测阑尾NET和腺癌生存结局的临床病理特征。比较了阑尾NET和腺癌组织学亚型的生存情况。
对2010年至2014年美国监测、流行病学和最终结果（SEER）数据库中18岁及以上的阑尾NET和腺癌患者进行回顾性分析。
NET亚组中女性与男性的比例为1.3比1，腺癌组中该比例为1.1比1。所有NET的诊断时平均年龄为50.3±17岁，腺癌为60.8±14.1岁。在NET亚组中，典型类癌（TC）、GCC、神经内分泌癌（NEC）和混合性神经内分泌-非神经内分泌癌（MANEC）的平均年龄分别为42.9±17.3岁、56.7±13.7岁、45.6±17.4岁和59.7±12.8岁。腺癌1年、2年、3年和4年总生存率分别为86.3%、73.5%、65.7%和57.6%。对于NET，TC的生存情况较好，1年和4年总生存率分别为97.4%和95.7%，而MANEC的生存结局最差，1年和4年总生存率分别为88.6%和62.2%。GCC的1年总生存率优于NEC（95.5%对92.9%），但4年总生存率略差（82%对84.8%）。诊断时年龄（风险比[HR]1.03）、非裔美国人种族（HR 1.47）和IV期疾病（HR 9.58）是阑尾腺癌生存的独立预测因素。对于NET，诊断时年龄较大、疾病分期较晚和非裔美国人种族被确定为生存的负面独立预测因素。
虽然先前的研究表明非典型NET（GCC、NEC和MANEC）更可能在更晚期出现，但本研究表明，大多数GCC、MANEC和NEC病例在I期和II期被诊断。另一方面，阑尾腺癌大多在IV期出现。就总生存期而言，NET的非典型组织学亚型的结局比TC差。虽然与腺癌相比，GCC、NEC和MANEC的总生存期较好，但在IV期疾病中这种优势消失，腺癌的1年和4年总生存率更高。需要开展提供详细治疗细节的前瞻性随机研究，以更好地确定阑尾NET的治疗方案。
