Radical resection in signet ring carcinoma of the ampulla of Vater: Report of an 11-year survivor

Carcinomas of the ampulla of Vater are uncommon tumors but have a better prognosis than typical pancreatic cancers. They tend to be well or moderately differentiated while the poorly differentiated variants are still quite aggressive. Signet ring features associated with poor differentiation traditionally confer a dismal prognosis in other gastrointestinal malignancies, particularly gastric cancer. Signet cell ring morphology has only been described in a few ampullary carcinomas with all cases reporting short follow-up. We describe a 58-year-old woman who presented with painless jaundice and a prominent ampulla of Vater by endoscopy. She underwent pancreaticoduodenectomy and was found to have a 1.0 x 0.8 cm poorly differentiated carcinoma with prominent signet ring cell features arising from the ampulla of Vater and invading into the periampullary duodenum but sparing the pancreatic parenchyma. No distant or nodal metastases were seen (pT2N0M0). No adjuvant therapy was given, and the patient remains free of disease with 134 months of follow-up.