Carcinoid of the ampulla of Vater: morphologic features and clinical implications

Abstract

Carcinoids involving the ampulla of Vater are rare lesions that may produce painless jaundice. The published data indicate that these tumors, in contrast to their midgut counterparts, metastasize in approximately half of cases irrespective of primary tumor size. Therefore, radical excision in the form of pancreaticoduodenectomy is recommended regardless of tumor size. As with other gastrointestinal carcinoid tumors, biological treatment with octreotide analogues can be applied to symptomatic patients. Tumor-targeted radioactive therapy is a newly emerging treatment option. We here report case of a carcinoid tumor of the ampulla of Vater presenting as painless jaundice in a 65-year old man and review the relevant literature, giving special attention to the morphologic features, clinical characteristics, and treatment modalities associated with this disease process.

Figure 1

The characteristic “double duct sign” on abdominal CT is indicative of obstruction at the level of the ampulla.

Figure 2

ERCP showing a markedly dilated CBD with an abrupt “shoulder” in the region of the ampulla, suggestive of a periampullary mass.

Figure 3

EUS identifying a 23 mm x 27 mm well circumscribed, round, hypoechoic mass in the region of the ampulla, which is distinct from the duodenal wall.