Ampullary Carcinoma: An Overview of a Rare Entity and Discussion of Current and Future Therapeutic Challenges

Curr Oncol. 2021 Sep 1;28(5):3393-3402. doi: 10.3390/curroncol28050293.


Ampullary carcinomas (ACs) represent a rare entity, accounting for approximately 0.2% of all gastrointestinal solid tumors and 20% of all periampullary cancers (PACs). Unfortunately, few data are available regarding the optimal therapeutic strategy for ACs due to their rarity, and physicians frequently encounter significant difficulties in the management of these malignancies. In this review, we will provide an overview of current evidence on AC, especially focusing on biological features, histological characteristics, and available data guiding present and future therapeutic strategies for these rare, and still barely known, tumors.

Keywords: adenocarcinoma; ampulla of Vater cancer; ampullary cancer; biliary tract cancer; chemotherapy.

Publication types

  • Review

MeSH terms

  • Adenocarcinoma*
  • Ampulla of Vater*
  • Common Bile Duct Neoplasms* / therapy
  • Humans