探讨壶腹部混合性神经内分泌-非神经内分泌肿瘤（MiNEN）临床表现和诊疗经验。

收集PubMed、Embase、万方、中国知网、维普数据库中已发表的有关壶腹部MiNEN的文献资料，检索时间为2021年3月31日。中文检索式为"混合性腺神经内分泌癌或混合性神经内分泌-非神经内分泌肿瘤+壶腹"；英文检索式为"mixed neuroendocrine-non-neuroendocrine neoplasms或MiNEN或mixed adenoneuroendocrine carcinoma或MANEC + ampulla or Vater"。

共纳入20篇文献24例患者，男女比例为1.3∶1，中位年龄63（37~89）岁。临床表现为黄疸15例，腹痛或腹部不适11例，体重减轻6例，恶心3例。肿瘤最大直径26（15~50）mm。患者接受CT检查15例，MRI、MRCP检查6例，ERCP检查6例，超声检查5例，内镜或超声内镜下穿刺活检9例。MiNEN诊断主要依靠肿瘤免疫组化检查，包括嗜铬粒蛋白A（CgA）、突触素（Syn）和CD56三种神经内分泌肿瘤标志物。胰十二指肠切除术11例，手术+化疗7例，姑息性治疗2例。患者1、3年生存率分别为69%和25%，中位生存时间仅为16个月。

壶腹部MiNEN是一类恶性程度高、预后差的罕见病例，术前诊断困难，黄疸症状、影像学及病理检查可能有助于明确诊断。早期手术及新辅助化疗有望改善预后。

To investigate clinical manifestations and diagnostic and treatment experience of ampullary mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN).

Literatures related to ampullary MiNEN were searched from PubMed, Embase, Wanfang Data, CNKI and Chongqing VIP databases from the inception date to March 31, 2021. The keywords included "mixed neuroendocrine-non-neuroendocrine neoplasms or MiNEN or mixed adenoneuroendocrine carcinoma or MANEC and ampulla or Vater".

20 articles consisting of 24 patients were searched. The male-to-female ratio was 1.3∶1, and the median age was 63(37-89) years. Clinical manifestations included jaundice in 15 cases, abdominal pain or abdominal discomfort in 11, weight loss in 6 and nausea in 3, respectively. The maximum tumor diameter was 26(15-50) mm. 15 patients received CT scan, 6 cases of MRI and MRCP, 6 cases of ERCP, 5 cases of ultrasound examination and 9 cases of endoscopic or endoscopic ultrasound biopsy, respectively. The diagnosis of MiNEN was mainly confirmed by immunohistochemical staining of neoplasms, including three neuroendocrine neoplasm markers, chromogranin A (CgA), synaptophysin (Syn) and CD56. Pancreatoduodenectomy was performed in 11 cases, surgery combined with chemotherapy in 7 and palliative treatments in 2, respectively. The 1- and 3-year survival rates were 69% and 25%. The median survival time was only 16 months.

Ampullary MiNEN is a rare disease with high-degree malignancy and poor prognosis. It is a challenging task to make preoperative diagnosis. Jaundice, imaging and pathological examination probably contribute to confirming the diagnosis. Prompt surgery and neoadjuvant chemotherapy are expected to improve the clinical prognosis.