A Rare Collision Tumor: Adenocarcinoma in the Ampulla of Vater and Neuroendocrine Tumor in the Lower Part of the Common Bile Duct

Abstract

In the biliary tree, only three cases of neuroendocrine tumor (NET) synchronous with adenocarcinoma have been reported so far. We experienced a case of NET, grade 2 (G2), of the common bile duct associated with adenocarcinoma of the ampulla of Vater (AoV). A Japanese man at his 60’s visited a local doctor, and obstructive jaundice was pointed out. Under the clinical diagnosis of carcinoma of the AoV, 20 x 20 mm, T3aN0M0 stage IIB, pylorus-preserving pancreaticoduodenectomy was performed. Dynamic computed tomography in an artery-dominant phase and microscopic examination revealed that the mass consisted of two different components; hypovascular, 2.5 cm-sized, exophytic adenocarcinoma in the AoV and hypervascular, 1.5 cm-sized, polypoid NET (G2) in the lower part of the common bile duct. The NET diffusely expressed neuroendocrine markers, including somatostatin receptor-2 (SSTR2), and adenocarcinoma cells arising from tubular adenoma focally expressed the neuroendocrine markers. Both malignancies were positive for caudal-type homeobox-2 (CDX2). It is presumed that NET occurred from intestinal-type adenoma/adenocarcinoma.