Primary signet ring cell carcinoma of ampulla of vater: Report of a case and a review of the literature.

Abstract Signet ring cell carcinoma of the ampulla of vater is a rare variant of adenocarcinoma. Morphology of the signet ring cell carcinoma is defined as intracytoplasmic mucin, with clear cytoplasm and hyperchromatic, eccentric nucleus. It is divided into three subtypes as intestinal, pancreatobiliary and mixed type according to immunohistochemical findings. Gastric type is mentioned as the fourth type by other publications. In this study, signet ring cell carcinoma of ampulla vater in a 60-year-old male patient is presented. Intracytoplasmic mucin accumulation was detected with PAS-Alcian Blue in cells that infiltrating the muscular wall of the duodenum. Immunohistochemically MUC1, MUC2, CK19, CK20, CDX2 positive; CK7 focal positive; MUC5AC locally weak positive detected in tumor cells. Because of coexistence MUC2, CK20, CDX2 antibodies associated with the intestinal type and CK7, CK19, MUC1 antibodies associated with pancreatobiliary type, the case was evaluated in favor of mixed type signet ring cell carcinoma of ampulla and discussed in terms of prognosis and treatment in the light of the literature. Key words: Ampulla of vater, carcinoma, signet ring cell