Summary

We describe a case report of a 53-year-old man with a 5-months history of progressive jaundice and upper abdominal pain. The patient was further evaluated and finally diagnosed with a high-grade ampullary neuroendocrine tumour (based on endoscopic-guided biopsy). Thereafter, he underwent pancreatoduodenectomy and adjuvant platinum-based chemotherapy. This extremely rare case presents his long-lasting disease-free survival compared with similar cases; this case report exemplifies a new, potentially efficient method for treating high-grade papillary neuroendocrine tumour and may pave the way for further clinical trials utilising this blueprint in the treatment of related conditions.