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

Shingo Yoshimachi, Hideo Ohtsuka, Takeshi Aoki, Takayuki Miura, Kyohei Ariake, Kunihiro Masuda, Masaharu Ishida, Masamichi Mizuma, Hiroki Hayashi, Kei Nakagawa, Takanori Morikawa, Fuyuhiko Motoi, Atsushi Kanno, Atsushi Masamune, Fumiyoshi Fujishima, Hironobu Sasano, Takashi Kamei, Takeshi Naitoh, Michiaki Unno

Research output: Contribution to journalArticlepeer-review

8 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)37-45
Number of pages9
JournalClinical Journal of Gastroenterology
Issue number1
Publication statusPublished - 2020 Feb 1


  • Ampulla of Vater
  • Case report
  • Literature review
  • Mixed adenoneuroendocrine carcinoma


Dive into the research topics of 'Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: a case report and literature review'. Together they form a unique fingerprint.

Cite this