背景:混合性腺神经内分泌癌(MANEC)是混合性神经内分泌非神经内分泌肿瘤(MiNEN)的一个亚组,被描述为含有神经内分泌和非神经内分泌双重成分的混合性肿瘤。这项研究的目的是评估MANEC在下胃肠道(GI)中的患病率,并提供可靠的生存率估计。
方法:根据PRISMA指南使用PubMed,Embase,Cochrane系统评价图书馆,WebofScience,和Scopus数据库,并进行贝叶斯分层生存汇总分析.
结果:在确定的182条唯一记录中,71项研究报告752例患者符合纳入标准。平均年龄为64.2±13.6,男女比例为1.25。总的来说,60.3%的MANEC位于附录中,在结肠中占29.3%,肛门直肠占10.4%。超过四分之一(29%)的患者在诊断时患有IV期疾病,阑尾的患病率高于结肠和肛肠原发性。超过80%的人具有高级(G3)内分泌成分。在152名患者中,中位随访20个月(四分位距限制,16.5-32),中位总生存期为12.3个月(95%可信间隔[95%CrI],11.3-13.7),1.12[95%CrI,0.67-1.83]转移性和非转移性MANEC之间的年龄调整风险比。与肛门直肠起源相比,在结肠病例中,诊断为IV期疾病在预后上更为不利。
结论:MANEC是一种临床侵袭性病理实体。这项研究的结果为了解下胃肠道内肿瘤的位置及其在总生存期方面的预后提供了新的见解。
BACKGROUND: Mixed adeno-neuroendocrine carcinomas (MANEC) are a subgroup of mixed neuroendocrine non-neuroendocrine neoplasms (
MiNEN) described as mixed neoplasms containing dual neuroendocrine and non-neuroendocrine components. The aim of this study was to appraise the prevalence of MANEC in the lower gastrointestinal (GI) tract and provide reliable estimates of survival.
METHODS: A systematic
review was undertaken in accordance with PRISMA guidelines using PubMed, Embase, Cochrane Library of Systematic
Review, Web of Science, and Scopus databases, and a Bayesian hierarchical survival pooled analysis was performed.
RESULTS: Of 182 unique records identified, 71 studies reporting on 752 patients met the inclusion criteria. Mean age was 64.2 ± 13.6, with a male-to-female ratio of 1.25. Overall, 60.3% of MANEC were located in the appendix, 29.3% in the colon, and 10.4% in the anorectum. More than a quarter (29%) of patients had stage IV disease at diagnosis, with higher prevalence in appendiceal than colonic and anorectal primaries. More than 80% had a high-grade (G3) endocrine component. Of the 152 patients followed up for a median of 20 months (interquartile range limits, 16.5-32), median overall survival was 12.3 months (95% credible interval [95%CrI], 11.3-13.7), with a 1.12 [95%CrI, 0.67-1.83] age-adjusted hazard ratio between metastatic and non-metastatic MANEC. Stage IV disease at diagnosis was more prognostically unfavorable in cases of colonic compared to anorectal origin.
CONCLUSIONS: MANEC is a clinically aggressive pathological entity. The results of this study provide new insights for the understanding of tumor location within the lower GI tract and its prognosis in terms of overall survival.