Aims and background: The aim of this report was to describe the way in which a rare and niche disease like ampulla of Vater carcinoma (AVC) was treated in real-world clinical practice.
Methods and study design: A retrospective analysis of consecutive patients with a diagnosis of AVC treated at our medical oncology unit between August 2004 and August 2013 was performed.
Results: We evaluated 8 consecutive patients with a median age of 60 years (range 56-84). At the last follow-up, 4 patients were alive without evidence of disease and 4 patients had died. The median follow-up time was 21.51 months (range 1-100.43), the median overall survival 23.19 months (range 7.07-102.2), and the median disease-free survival 18.26 months (range 0-102.2). Six patients underwent surgery, which consisted of pylorus-preserving pancreaticoduodenectomy, R0 in all cases. Tumor histology was adenocarcinoma in all patients. Two patients presented with locally advanced disease. Only 1 patient presented with metastases while 3 patients subsequently developed metastases. Two patients received chemotherapy for metastatic disease; in both cases disease progression was observed at the first disease evaluation.
Conclusions: We can consider AVC as a pathology niche and pancreaticoduodenectomy as the effective treatment for these patients.