Secondary tumours of the ampulla of Vater are rare. Underlying primary tumours, clinical presentation, macroscopic appearance, treatment strategies and outcome of secondary ampullary lesions have not been systematically analysed. The present case study reported a 57-year old patient with an ampullary metastasis from renal cancer and a literature review was performed in which a further 32 patients were included. The most common responsible primary tumours were malignant melanoma and renal clear cell carcinoma, followed by breast cancer. The time interval between the diagnosis of the primary tumour and the ampullary metastasis was highly variable, and may be as long as 10 years, particularly for renal cancer. Patients may present with unspecific abdominal discomfort, jaundice or upper gastrointestinal bleeding. The gross appearance was largely indistinguishable from that of a primary tumour. Lesions may present as polypoid or irregular, soft and friable tumour mass, in certain cases with superficial ulceration. In ~50% of cases, the ampullary metastasis was the only metastatic lesion, while in the remaining cases, the cancer had spread to one or more organs. The prognosis was generally poor. The management requires a multi-modal approach, including endoscopic, surgical and oncological procedure.
Keywords: ampulla of Vater; ampullary tumour; differential diagnosis; histology; metastasis; secondary tumour.