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

Bum Chul Kim; Tae Jun Song; Hyuk Lee; Mee Joo; Won Ki Bae; Nam-Hoon Kim; Kyung-Ah Kim; June Sung Lee

doi:10.4166/kjg.2013.62.5.301

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

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

Case Report

Korean J Gastroenterol 2013;62(5):301-305.

Published online: 4 October 2013

DOI: https://doi.org/10.4166/kjg.2013.62.5.301

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

Bum Chul Kim, Tae Jun Song, Hyuk Lee, Mee Joo¹, Won Ki Bae, Nam-Hoon Kim, Kyung-Ah Kim, June Sung Lee

Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea

¹Department of Pathology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea

Correspondence to: Tae Jun Song, Department of Internal Medicine, Inje University Ilsan Paik Hospital, 170 Juhwa-ro, Ilsanseo-gu, Goyang 411-706, Korea. Tel: +82-31-910-7200, Fax: +82-31-910-7219, E-mail: tjsong@paik.ac.kr

Received 17 January 2013 Revised 5 April 2013 Accepted 10 April 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Neuroendocrine tumors, Biliary tract, Klatskin's tumor

References

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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
							trisegmentectomy
							Roux‐ eu‐ Y left
							hepaticojejunostomy
4	Sankary et al.,⁸ 1995	47	F	Jaundice	No	Carcinoid, low grade	Roux‐ eu‐ Y
							hepaticojejunostomy
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
							triseqmentectomy,
							CBD resection, portal
							LN dissection
8	Schmitt et al.,¹¹ 2008	32	F	RUQ pain	Liver, pancreas, LN	Carcinoid, low grade	Roux‐ eu‐ Y
							hepaticojejunostomy
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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