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Abstract

Cholangiocarcinoma is an uncommon adenocarcinoma with poor prognosis. Signet ring cell cholangiocarcinoma is an extremely rare disease, with only 11 cases reported to date. However, its incidence is increasing. Interestingly, there is increased incidence of other SRC tumors (colon, gastric, etc.) as well. Most cases have described a localized mass of signet ring cells, most commonly in the common bile duct. In our report, a 69-year-old Caucasian man presented with jaundice and abdominal pain of three days. The patient underwent a CT scan, ERCP, and EUS as part of his initial workup. Biopsies were obtained during EUS from the CBD disclosing signet ring cholangiocarcinoma. ERCP showed a diffusely infiltrative tumor of both left and right hepatic ducts. This appears to be the first description of diffuse type signet ring cell cholangiocarcinoma. Unfortunately, the patient experienced rapid clinical deterioration which precluded treatment. Survival was extremely short-the patient passed away three weeks after initial appearance of symptoms. As the incidence of SRC's in general, as well as the incidence of SRC cholangiocarcinoma appear to be on the rise, more cases are likely to be encountered. If our observation of the extremely virulent presentation of diffuse-type SRC cholangiocarcinoma is confirmed, perhaps palliative care only should be considered in these patients at the onset.