Journal List > Korean J Gastroenterol > v.62(5) > 1007163

Kim, Song, Lee, Joo, Bae, Kim, Kim, and Lee: A Case of Small Cell Neuroendocrine Tumor Occurring at Hilar Bile Duct


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.


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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.
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.
Fig. 3.
Endoscopic retrograde cholangiogram reveals malignant stricture of the common hepatic duct.
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).
Table 1.
Data on Hilar Neuroendocrine Tumors
Case No Study Age (yr) Sex Clinical symptom Metastases Pathology Treatment
1 Judge et al.,5 1976 19 M Jaundice RUQ pain Liver, pancreas, LN Small cell carcinoma, high grade Exploratory laparotomy
2 Brown et al.,6 1990 35 F Jaundice No Atypical carcinoid, Roux‐ eu‐ Y
            intermediate grade hepaticojejunostomy
3 Gembala et al.,7 1993 28 M Jaundice Liver Carcinoid, low grade Right hepatic
              Roux‐ eu‐ Y left
4 Sankary et al.,8 1995 47 F Jaundice No Carcinoid, low grade Roux‐ eu‐ Y
5 Chamberlain and 37 F Itching No Carcinoid, low grade Roux‐ eu‐ Y
  Blumgart,9 1999           hepaticojejunostomy
6 Chamberlain and 67 F Itching No Carcinoid, low grade Roux‐ eu‐ Y
  Blumgart,9 1999           hepaticojejunostomy
7 Ferrone et al.,10 2007 52 M Elevated LFT No Carcinoid, low grade Right hepatic
              CBD resection, portal
              LN dissection
8 Schmitt et al.,11 2008 32 F RUQ pain Liver, pancreas, LN Carcinoid, low grade Roux‐ eu‐ Y
9 Park et al.,12 2011 73 F RUQ pain LN Large cell carcinoma, Roux‐ eu‐ Y left
            high grade hepaticojejunostomy

M, male; F, female; RUQ, right upper quadrant; LN, lymph nodes; LFT, liver function test; CBD, common bile duct.

Table 2.
Reported Cases of Small Cell Neuroendocrine Tumors of Bile Duct in Korea
Case No Study Age (yr) Sex Location Survival duration Treatment
1 Kim et al.,15 2000 64 M CBD Unknown Pancreaticoduodenectomy
2 Kim et al.,16 2004 57 M CBD 9 months Pancreaticoduodenectomy+adjuvant RT
3 Park et al.,17 2004 60 F Proximal CBD 5 months Transhepatic biliary drainage
            Roux‐ en‐ Y hepaticojejunostomy
4 Jeon et al.,18 2006 65 M Proximal CBD 1 year Pancreaticoduodenectomy
5 Cho et al.,19 2009 59 M Distal CBD 6 months Bile duct tumor resection
            Roux‐ en‐ Y hepaticojejunostomy

M, male; F, female; CBD, common bile duct; RT, radiotherapy.

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