背景:混合神经内分泌和非神经内分泌组织学肿瘤被归类为碰撞,合并,或苯丙胺,可以发生在大多数器官,包括肝胰胆管.鉴于Vater壶腹混合性腺神经内分泌癌(MANEC)的罕见,病人的特点,管理,和结果仍不清楚。我们试图系统地回顾关于壶腹MANEC的全球文献。
方法:通过系统搜索MEDLINE(通过PubMed)确定合格研究,Scopus,和Cochrane图书馆数据库(搜索结束日期:1月5日,2022),根据PRISMA2020声明。
结果:共39项研究报告56例壶腹MANEC患者。中位年龄为63.0(四分位距[IQR]:51.0-69.0)岁,男性占55.6%(n=25/45)。大多数患有合并肿瘤(64.4%;n=29/45),其次是碰撞(24.4%;n=11/45),和苯丙胺肿瘤(11.1%;n=5/45)。超过一半的淋巴结转移(56.8%;n=25/44),然而,只有7.9%有远处转移(n=3/38).肿瘤切除(即,主要是胰十二指肠切除术)的发生率为96.3%(n=52/54),其次是辅助化疗61.8%(n=21/34)。近一半的患者经历了疾病复发(47.2%;n=17/36),中位随访时间为12.0(IQR:3.0-16.0)个月,42.1%(n=16/38)在中位随访时间12.0(IQR:4.0-18.0)个月内死亡。最常见的死亡原因是疾病进展/复发,占81.3%(n=13/16)。
结论:壶腹部MANEC的早期诊断和治疗具有挑战性,但对于改善预后至关重要,因为许多患者被诊断为晚期疾病,并且预后不良。多中心颗粒数据是必要的,以进一步了解和改善这些患者的结果。
Tumors of mixed neuroendocrine and nonneuroendocrine histology are classified as collision, combined, or amphicrine and can occur in most organs, including the hepato-pancreato-biliary tract. Given the rarity of mixed adenoneuroendocrine carcinoma (
MANEC) of the ampulla of Vater, the patient characteristics, management, and outcomes remain unclear. We sought to systematically review the worldwide literature on ampullary MANECs.
Eligible studies were identified through a systematic search of the MEDLINE (via PubMed), Scopus, and Cochrane Library databases (end-of-search-date: January 5th, 2022), according to the PRISMA 2020 statement.
A total of 39 studies reporting on 56 patients with ampullary
MANEC were included. The median age was 63.0 (interquartile range [IQR]: 51.0-69.0) years and 55.6% were male (n = 25/45). Most had combined tumors (64.4%; n = 29/45), followed by collision (24.4%; n = 11/45), and amphicrine tumors (11.1%; n = 5/45). More than half had lymph node metastasis (56.8%; n = 25/44), yet only 7.9% had distant metastasis (n = 3/38). Tumor resection (i.e., mostly pancreaticoduodenectomy) was performed in 96.3% (n = 52/54), followed by adjuvant chemotherapy in 61.8% (n = 21/34). Nearly half experienced disease recurrence (47.2%; n = 17/36) over a median follow-up of 12.0 (IQR: 3.0-16.0) months, and 42.1% (n = 16/38) died over a median follow-up of 12.0 (IQR: 4.0-18.0) months. The most common cause of death was disease progression/recurrence in 81.3% (n = 13/16).
Early diagnosis and management of ampullary
MANEC is challenging yet crucial to improve outcomes since many patients are diagnosed at an advanced disease stage and have unfavorable outcomes. Multicenter granular data are warranted to further understand and improve outcomes in these patients.