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.