Invasive micropapillary carcinoma of ampulla of Vater in a young female with negative lymph nodes: A case report and review of literature

Invasive micropapillary carcinoma (IMPC) is an extremely rare and aggressive subtype of adenocarcinoma that was first described in breast cancer. It is characterised by an inside-out growth pattern with frequent lymphatic spread. Only few cases are described in the rare ampullo-pancreatobiliary location. In this case report, the diagnosis was unexpected given the patient’s age and gender, as well as the negative nodal status. Despite the reported aggressive behaviour of this tumour, no optimal treatment strategy for IMPC in the ampulla of Vater has been established. In addition, this variant is not included in the recent WHO of the digestive system.