Adenocarcinoma of the ampulla of Vater is a biliary tumour arising from the distal biliary epithelium at the ampulla of Vater. It is a relatively rare tumour, accounting for approximately 7% of all periampullary malignancies. Leptomeningeal metastasis, a complication occurring in 5% of all cancer patients, is a feature very seldomly reported in this type of malignancy. The prognosis is very poor, as current treatment options for leptomeningeal metastasis are limited by low efficacy and high toxicity. This study presents the case of a patient with an ampullary adenocarcinoma, who first was treated with surgical resection for localised disease. However, the disease evolved and palliative chemotherapy was started when colonic metastases became evident. Twelve months after diagnosis, leptomeningeal metastases were detected. To our knowledge, only one similar case has been reported previously, slightly different from the type of ampullary carcinoma presented in this study. Leptomeningeal metastasis should always be considered in a patient presenting with neurologic signs and a history of an ampullary malignancy.

(BELG J MED ONCOL 2022;16(3):125–7)