Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: a case report and literature review

Clin J Gastroenterol. 2020 Feb;13(1):37-45. doi: 10.1007/s12328-019-01009-2. Epub 2019 Jul 24.


Mixed adenoneuroendocrine carcinoma (MANEC) is defined as a tumor composed of both adenocarcinoma and neuroendocrine components. Here, we report the case of a 75-year-old woman with ampullary MANEC. She visited a physician with the chief complaint of dark urine and was diagnosed with advanced jaundice. Subsequently, she was referred to our hospital. Contrast-enhanced computed tomography scan revealed a neoplastic lesion measuring approximately 2 cm with a contrast effect at the duodenal papilla. Upper endoscopy showed a non-exposed tumor at the duodenal papilla. After biliary drainage, a subtotal stomach-preserving pancreaticoduodenectomy was performed. Histopathological examination revealed that the tumor components were composed of circular-to-oval atypical cells admixed with tubular adenocarcinoma tissue. These atypical cells were immunohistochemically positive for synaptophysin and diagnosed as neuroendocrine carcinoma with a Ki-67 labeling index of 63%. The patient was diagnosed with MANEC with a neuroendocrine carcinoma component of approximately 40%. The neuroendocrine carcinoma component had metastasized to the posterior pancreatic lymph nodes. Despite starting adjuvant chemotherapy with S-1, computed tomography revealed the presence of multiple liver metastases within 4 months after surgery. MANEC with neuroendocrine carcinoma is well known to have an extremely poor prognosis. Therefore, establishing a multidisciplinary therapy including chemotherapy is crucial.

Keywords: Ampulla of Vater; Case report; Literature review; MANEC; Mixed adenoneuroendocrine carcinoma.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adenocarcinoma / complications
  • Adenocarcinoma / diagnosis
  • Adenocarcinoma / pathology*
  • Adenocarcinoma / surgery
  • Aged
  • Ampulla of Vater*
  • Carcinoma, Neuroendocrine / complications
  • Carcinoma, Neuroendocrine / diagnosis
  • Carcinoma, Neuroendocrine / pathology*
  • Carcinoma, Neuroendocrine / surgery
  • Cholangiopancreatography, Endoscopic Retrograde
  • Common Bile Duct Neoplasms / complications
  • Common Bile Duct Neoplasms / diagnosis
  • Common Bile Duct Neoplasms / pathology*
  • Common Bile Duct Neoplasms / surgery
  • Female
  • Humans
  • Jaundice, Obstructive / etiology
  • Mixed Tumor, Malignant / complications
  • Mixed Tumor, Malignant / diagnosis
  • Mixed Tumor, Malignant / pathology*
  • Mixed Tumor, Malignant / surgery
  • Pancreaticoduodenectomy
  • Tomography, X-Ray Computed