Primary neuroendocrine tumor of the left hepatic duct: a case report with review of the literature.
ABSTRACT Primary Biliary Tract Neuroendocrine tumors (NET) are extremely rare tumors with only 77 cases been reported in the literature till now. We describe a case of a left hepatic duct NET and review the literature for this rare malignancy. To the best of our knowledge the present case is the first reported case of a left hepatic duct NET in the literature. In spite of availability of advanced diagnostic tools like Computerized Tomography (CT) Scan and Endoscopic Retrograde Cholangio Pancreaticography (ERCP) a definitive diagnosis of these tumors is possible only after an accurate histopathologic diagnosis of operative specimens with immunohistochemistry and electron microscopy. Though surgical excision remains the gold standard treatment for such tumors, patients with unresectable tumors have good survival with newer biologic agents like Octreotride.
- Journal of the American College of Surgeons 03/2008; 206(2):399. · 4.50 Impact Factor
Article: Primary common bile-duct carcinoid.British Journal of Surgery 03/1968; 55(2):147-9. · 4.84 Impact Factor
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ABSTRACT: Primary carcinoid tumors of the extrahepatic biliary tree are exceedingly rare, accounting for 0.2-2% of all digestive carcinoids. The authors in this study describe a case of biliary duct primary well-differentiated endocrine tumor in a 30-year-old man with symptoms of biliary obstruction and watery diarrhoea. Abdominal ultrasound showed a 2-cm solid lesion in the head of the pancreas, compressing the distal common bile duct. A computed tomography scan confirmed these findings, revealing the hypervascular pattern of the tumor. Gastrointestinal hormonal screening demonstrated an increase in plasma serotonin. The patient underwent standard pylorus-preserving pancreatoduodenectomy. Pathological examination showed a neuroendocrine tumor of the distal common bile duct measuring 1.8 cm in greatest dimension. The tumor cells were immunopositive for neuron-specific enolase (NSE), chromogranin A, synaptophysin, serotonin, and cytokeratin. Stains for gastrin and somatostatin were negative. Seven years later, the patient is well, with no evidence of disease. Given the site of these tumors and the difficulty in differentiating them from periampullary lesions, decisions as to the appropriate surgical approach may be problematic. After an exhaustive review of the literature, the authors conclude that pancreatoduodenectomy is the treatment of choice.Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin 08/2006; 449(1):104-11. · 2.68 Impact Factor
Hindawi Publishing Corporation
Case Reports in Surgery
Volume 2012, Article ID 786432, 7 pages
Primary NeuroendocrineTumorof the LeftHepatic Duct: ACase
AjayH.Bhandarwar,Taher A.Shaikh,Ashok D.Borisa,JaydeepH.Palep,
Division of GI and HPP Surgery, Department of Surgery, Grant Medical College & Sir JJ Group of Hospitals, Byculla,
Mumbai 400008, India
Correspondence should be addressed to Ajay H. Bhandarwar, firstname.lastname@example.org
Received 29 April 2012; Accepted 29 August 2012
Academic Editors: T. C ¸olak and M. Ganau
Copyright © 2012 Ajay H. Bhandarwar et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
Primary Biliary Tract Neuroendocrine tumors (NET) are extremely rare tumors with only 77 cases been reported in the literature
till now. We describe a case of a left hepatic duct NET and review the literature for this rare malignancy. To the best of our
knowledge the present case is the first reported case of a left hepatic duct NET in the literature. In spite of availability of advanced
diagnostic tools like Computerized Tomography (CT) Scan and Endoscopic Retrograde Cholangio Pancreaticography (ERCP)
a definitive diagnosis of these tumors is possible only after an accurate histopathologic diagnosis of operative specimens with
immunohistochemistry and electron microscopy. Though surgical excision remains the gold standard treatment for such tumors,
patients with unresectable tumors have good survival with newer biologic agents like Octreotride.
NET is derived from the embryonal neural crest cells called
Argentaffin or Kulchitsky cells and have a potential for
secreting serotonin. This tumor can arise anywhere in the
distribution of the Argentaffin cell system. In addition to
the most common sites of occurrence, namely, ileum and
appendix these tumors have reported to occur in bladder,
prostate, rectum, stomach, bronchi, pancreas, and biliary
Primary Biliary Tract NETs are rare and account for
0.2%–2% [1, 2] of all gastrointestinal neuroendocrine
The Literature documents about 77 cases of neuroen-
docrine tumor arising from the biliary tree which includes
common bile duct, common hepatic duct, cystic duct, and
We report a case of Primary Biliary NET arising from the
left hepatic duct. An extensive search of the literature has
yielded no reference regarding a NET arising from the left
Hepatic ducts. The present case is the first reported case of a
NET arising in the left hepatic duct.
A 69-years-old female presented with colicky pain in the
right hypochondrium since 3 years. She had past history of
open cholecystectomy done for gall stones 15 years back.
On physical examination the patient was anicteric with
soft abdomen. An Ultrasonography (USG) of the abdomen
showed a hypoechoic lesion of size 3.5cm × 4cm in
segment 4 of the liver. Computerized Tomography (CT)
of the abdomen showed a 4.1 × 3.7cm heterogeneously
enhancing mass lesion in segment IV of liver abutting the
left branch of portal vein (Figure 1). A Magnetic Resonance
Imaging (MRI) of upper abdomen with Magnetic Resonance
CholagioPancreaticography (MRCP) showed a filling defect
in the left hepatic duct with lesion in the adjacent part of
liver in segment IV, suggestive of a left hepatic duct tumor
with infiltration in the liver (Figure 2). Tumor marker serum
alfa fetoprotein was mildly raised. Patient was worked up
for left hepatectomy. Intraopertaively a lesion arising from
the left hepatic duct involving the left branch of portal
vein and extending upto the portal confluence was found
(Figure 3) rendering the tumor unresectable. A biopsy was
2Case Reports in Surgery
Figure 1: CT scan showing mass lesion in segment 4 abutting the
left branch of portal vein.
Figure 2: MRCP showing a filling defect in the left hepatic duct.
Figure 3: Intraoperative picture showing tumor arising from the
left hepatic duct. CBD hooked in yellow, hepatic artery in red, and
portal vein in blue.
taken from the mass and procedure was abandoned in
view of inoperability. Histopathology showed typical rosette
appearance of a neuroendocrine tumor (Figure 4) and
immunohistochemistry positive for CD56, Chromogranin
and Synaptophysin (Figure 5). Ultrastructural study of the
of multiple neurosecretory granules with muscle tissue.
A whole body Positron Emission Tomography (PET)
Figure 4: Histopathology showing a typical rosette appearance of
Figure 5: Immunohistochemistry staining positive for Chromo-
granin (a) and Synaptophysin (b).
somatostatin receptor expressing lesion in the hepatobiliary
system (Figure 7).
The patient was started on long-acting Octreotride
therapy single dose every month. The patient has received
12 of such doses and is doing well after a 1-year followup
without any complications. A follow up MRI (Figure 8)
upper abdomen with MRCP at 1 year showed the absence of
filling defect in the Left hepatic duct that was seen previously
which showed tumor regression.
Case Reports in Surgery3
767-10-a 80 kV x30000
Figure 6: Electron microscopy picture showing multiple granules of varying sizes.
Figure 7: PET Scan (a) and Octreotride labelled (b) scan showing
tumor limited to the hepatobiliary system.
Figure 8: Follow up MRCP at 1 year showing absence of any filling
defect in the left hepatic duct.
Primary Biliary-tract NETs are very rare. They account for
0.2–2% of all gastrointestinal NET [1, 2] reason being the
paucity of enterochromaffin cells from which NETs arise in
this area. Chronic inflammation of the bile duct epithelium
is responsible for metaplasia of these enterochromaffin cells
and formation of NET.
Davies  in 1959 reported NET of the distal bile
duct and pancreatic duct which represented more of a
periampullary NET rather than a biliary tract. Pilz  in
1961 was credited to report the first case of a Biliary Tract
Biliary Tract NET have been reported so far in the literature
since 1961 (Table 1).
Till now no NET in the literature has been reported in
the isolated left hepatic duct possibly making our case the
first reported case of an isolated left hepatic duct NET.
4 Case Reports in Surgery
Table 1: Showing study of reported cases of Biliary tract NET.
Pilz,  1961
Little et al., 
(3)Bergdahl,  197679F
Judge et al., 
Vitaux et al., 
Nakamuara et al.
Abe et al., 
Goodman et al.,
Jutte et al., 
Popescu,  1986
Alexander et al.,
Gastinger et al.
Reinhardt et al.
Chittal and Ra,
Fujita et al., 
Ross  1989
Brown et al., 
(19)Bumin et al., 
(9)28FRUQ PainCystic duct
(12)64F Hematemesis CBD
46FRUQ painCystic duct
(20)Fellows et al. 
(21) Beszny´ ak et al. 
al.,  1991
et al.,  1991
Newman et al.,
(25)Dixon et al., 1992
60FRUQ pain CBD
Rugge et al., 
Gembala et al.,
et al.,  1995
(29)Sankary et al., 
Table 1: Continued.
No. Case reference
Hao et al., 
Kopelman et al.,
Belli et al., 
Bembenek et al.,
Nahas et al., 
Ross et al., 
Hermina et al.,
(39)Perakath et al. 
(30) 47M CBD
69MRUQ painCystic duct
Chan et al 
(41)Maitra et al., 
(42)Maitra et al., 
Juturi et al., 
(44)Turri´ on et al., 
Pawlik et al., 
(46)Podnos et al., 
(47)Podnos et al., 
Volpe et al., 
(49) Menezes et al., 
Ligato et al., 
(51)Hubert et al., 
(52)Hubert et al., 
(53)Hubert et al., 
Nesi et al., 
Kim et al., 
(56)Caglikulekci et al.,
(57)Honda et al., 
(55) 67FJaundice CBD
Case Reports in Surgery5
Table 1: Continued.
(58)Todorki et al., 
Case referenceAge Sex Complaint
Sethi et al., 
(60)Stavridi et al., 
NA NANACystic duct
(61)Jim´ enez et al., 
(62)Ferrone et al., 
NA NANA NA
Nafidi et al., 
(64)Gusani et al., 
NANA NA CBD
(65)Schmitt et al., 
NANA NA Hilar
Costantini et al.,
Felekouras et al.,
Price et al., 
Price et al., 
Price et al., 
Tonnhofer et al.,
Zhan et al. 
(73)Squillaci et al., 
60FJaundice Cystic duct
NA NA Jaundice CBD
NANA Jaundice Hilar
(74)Squillaci et al., 
Tsalis et al., 
(76) Lee et al.,  2011
al.,  2011
(78)Present case69F Pain
bile duct bifurcation.
The most common site of malignancy in the biliary
tract was common bile duct (57.14%) followed by the
hilar confluence (27.28%), the cystic duct (9.1%), common
hepatic duct (5.12%) and finally the left hepatic duct
The mean age of presentation was 47 years (range 6 years
to 79 years).
The male to female ratio is 1:1.23 showing that the
biliary NET has a preponderance for female.
By far the most common symptom in patients of Biliary
tract NET is Jaundice (63.4%) followed by Pain (14.1%),
jaundice with pain (12.7%) and remaining nonspecific
symptoms like weight loss.
The incidence of a Carcinoid syndrome in patients of
Biliary Tract NET is very rare. Only 4 cases which include
a single case published by Nesi et al.  in 2006 with
symptoms of diarrhea due to secretion of serotonin and 3
cases by Price et al.  in 2009 with features of Zollinger
Ellison syndrome due to secretion of gastrin from tumor in
Biliary Tract NET are rare tumors that typically present with
jaundice and pain. As compared to its counterpart Cholan-
giocarcinoma Biliary NET occurs in a younger age group
with a female preponderance . Biliary NET usually are
nonsecreting tumor. Preoperative diagnosis of these tumors
ical diagnosis which must include a immunohistochemistry
study and electron microscopy. Biliary tract NET are slow-
growing indolent tumor which have a limited propensity
for local and metastatic spread. Surgical resection aimed
at complete tumor excision with bilio-enteric continuity
offers the best cure and high survival rates. Patients who
have undergone resection have a long term survival. Even
in inoperable patients chemotherapy with newer biologic
agents like Octreotride have a favorable outcome on the
Conflict of Interests
The authors declare that they have no conflict of interest.
We would like to thank Padmashree Dr. T. P. Lahane Dean
of Grant Medical College Mumbai for the support and
encouragement provided to publish this paper. We would
also like to thank Dr. Sanjay Navani and Dr. M. B. Tayade
Head of department General Surgery Grant Medical College
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