Signet ring cell carcinoma of the ampulla of Vater: case series

Signet ring cell carcinomas (SRCC) are aggressive neoplasms with poor prognoses, commonly found in the stomach, colon, and small intestine. However, their occurrence in the Ampulla of Vater is rare and their origin remains unclear. Among 163 cases of surgically treated ampullary carcinomas, we observed 4 cases (2.4%) of SRCC with jaundice being the primary clinical manifestation, followed by weight loss and abdominal pain. Duodenoscopy played a pivotal role in the diagnosis. Anatomopathological findings are detailed, emphasizing immuno-histological lineage and lymph node involvement. Two cases underwent adjuvant treatment (GemCap and Folfirinox), with follow-up periods ranging from 18 to 64 months. We review the literature, highlighting the importance of radical surgery, lymph node involvement, and systemic treatment in the survival of these patients.