Korean J Gastroenterol.  2013 Nov;62(5):301-305. 10.4166/kjg.2013.62.5.301.

A Case of Small Cell Neuroendocrine Tumor Occurring at Hilar Bile Duct

• Kim BC
• Song TJ
• Lee H
• Joo M
• Bae WK
• Kim NH
• Kim KA
• Lee JS

• KMID: 1775739
• DOI: http://doi.org/10.4166/kjg.2013.62.5.301

Abstract

Neuroendocrine tumors of the extrahepatic biliary tree are extremely rare malignancies accounting for 0.2-2.0% of all gastrointestinal carcinoid tumors. Neuroendocrine tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively and nearly impossible to differentiate from cholangiocarcinoma. Statistically, the most common anatomic location in the biliary tree is the common bile duct, followed by the perihilar region. Herein, we present a case of a small cell neuroendocrine carcinoma of the hilum in a 79-year-old man following laparotomy. To our knowledge, this is the first case of small cell type neuroendocrine carcinoma of hilar bile duct reported in Korea.

Keyword

Neuroendocrine tumors; Biliary tract; Klatskin's tumor

MeSH Terms

Aged
Bile Duct Neoplasms/*diagnosis/pathology/surgery
Cholangiopancreatography, Endoscopic Retrograde
Diagnosis, Differential
Hepatic Duct, Common/pathology
Humans
Male
Neuroendocrine Tumors/*diagnosis/pathology/surgery
Tomography, X-Ray Computed

Figure

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  Fig. 1. (A, B) Contrast-enhanced CT image shows a diffusely infiltrating soft-tissue mass at the hilum (arrows) that extends along the gallbladder neck and cystic duct.

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  Fig. 2. Magnetic resonance cholangiopancreatography demonstrates low signal intensity mass involving the hepatic duct confluence (arrow). Both the right and left hepatic ducts are dilated.

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  Fig. 3. Endoscopic retrograde cholangiogram reveals malignant stricture of the common hepatic duct.

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  Fig. 4. Histopathological findings. (A) The tumor cells are composed predominantly of small to medium-sized round or oval cells with hyperchromatic nuclei, inconspicuous nucleoli, and scanty cytoplasm (H&E, ×400). (B) Tumor cells are stained positive for synaptophysin immunohistochemistry, which indicates the presence of a neuroendocrine tumor (×200).

Reference

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