|Year : 2014 | Volume
| Issue : 2 | Page : 100-103
The dilemma of imaging in the diagnosis and management of cholangiocarcinoma: Report of a case and review of literature
Philip Oluleke Ibinaiye1, Abiodun Oludotun Adeyinka2, Adefemi Oladiran Afolabi3, Ayorinde David Dawotola4
1 Department of Radiology, Ahmadu Bello University Teaching Hospital, Kaduna State, Zaria, Nigeria
2 Department of Radiology, University College Hospital, Ibadan, Nigeria
3 Department of Surgery, University College Hospital, Ibadan, Nigeria
4 Department of Radiotherapy and Oncology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
|Date of Submission||30-Jul-2013|
|Date of Acceptance||30-Sep-2013|
|Date of Web Publication||16-Jul-2014|
Philip Oluleke Ibinaiye
Department of Radiology, Ahmadu Bello University Teaching Hospital, Zaria
A case of cholangiocarcinoma (CC) of the distal common bile duct in a 59-year-old woman in whom an ultrasound and CT diagnosis of mass in the head of pancreas was made but, surgery and histology revealed the correct diagnosis of CC. This case highlighted the dilemma of imaging in the diagnosis and management of cholangiocarcinoma. The clinical presentation, radiological diagnosis, and literature review have also been discussed.
Keywords: Cholangiocarcinoma, dilemma, imaging
|How to cite this article:|
Ibinaiye PO, Adeyinka AO, Afolabi AO, Dawotola AD. The dilemma of imaging in the diagnosis and management of cholangiocarcinoma: Report of a case and review of literature. Sub-Saharan Afr J Med 2014;1:100-3
|How to cite this URL:|
Ibinaiye PO, Adeyinka AO, Afolabi AO, Dawotola AD. The dilemma of imaging in the diagnosis and management of cholangiocarcinoma: Report of a case and review of literature. Sub-Saharan Afr J Med [serial online] 2014 [cited 2019 Oct 16];1:100-3. Available from: http://www.ssajm.org/text.asp?2014/1/2/100/136823
| Introduction|| |
Cholangiocarcinomas (CC) are malignancies of the biliary duct system, originating in the liver and terminating at the ampulla of Vater.  CC are encountered in three anatomic regions, namely, intrahepatic, extra-hepatic (Perihilar) and distal extra-hepatic. Perihilar tumors are the most common, and intra-hepatic tumors are the least common.  Perihilar tumors, also called Klatskin tumors (after Klatskin's description of them in 1965), occur at the bifurcation of the right and left hepatic ducts. 
Each year, 2,500 cases occur in the United States with average incidence of 1 case per 100,000 people. , Internationally, the incidence in Western countries ranges from 2 to 6 per 100,000 people with the highest incidence in Japan at 5.5 per 100,000 people and in Israel at 7.3 per 100,000 people.  The incidence in Nigeria could however not be ascertained in an extensive search through literature. The male to female ratio is 1.5:1 and the highest incidence occurs in patients aged approximately 60 years. 
CC is a rare tumor and the purpose of this case report is to present a case of CC masquerading as tumor of the head of pancreas on ultrasound and CT so that it will raise the index of suspicion to the radiologist, that despite the infrequent occurrence of CC, its diagnosis should be considered in every case of obstructive jaundice, and to emphasize that carcinoma of the distal common bile duct presents diagnostic and therapeutic dilemmas similar to carcinoma of the head of the pancreas.
| Case report|| |
A.O. was a 59-year-old female administrator with a broadcasting firm in Ibadan. She presented at the surgical outpatient clinic via the general outpatient clinic of University College Hospital, Ibadan on account of 7-week history of jaundice, pruritus and weight loss. There was also history of passage of pale stools, dark-colored urine and post-prandial vomiting all of six weeks duration. However, there was no history of hepatitis, no previous blood transfusion or significant alcohol intake. The past medical history revealed that she was treated for pulmonary tuberculosis in this same hospital 6 years ago. She was also a known hypertensive for 3 years and this was controlled by oral moduretic. She had bleeding hemorrhoids about 2 years ago which was treated. Family and social history were not contributory.
On examination, she was found to be obese, chronically ill-looking, deeply jaundiced with generalized scratch marks and skin excoriation. However, she was not pale or febrile and there was no peripheral lymphadenopathy. Abdominal examination showed hepatomegaly but, no area of tenderness or ascites. The gall bladder was not palpable. The digital rectal examination showed anal tags at 5 and 7 o'clock. No rectal mass was seen. The examining finger was stained with pale stool. Other systems were essentially normal.
The provisional diagnosis included obstructive jaundice secondary to carcinoma of the head of the pancreas with periampullary carcinoma as differential diagnosis.
The liver function tests showed elevated total and direct (conjugated) bilirubin, 29 and 25.8 mg/dl, respectively. However, the liver enzymes, proteins and albumin were within normal limits. The full blood count and the clotting profiles were also within normal limits.
Abdominal ultrasound showed a mass of mixed echogenicity (isoechoic and hypoechoic) in the region of the head of pancreas with dilated intrahepatic ducts. There was mild/moderate ascites. The gall bladder and other findings were essentially normal.
Abdominal CT showed dilatation of the intra and extra-hepatic bile ducts down to the level of the pancreatic head. No mass lesion was seen in the liver. An area of ill-defined heterogeneous mass lesion was noted within the pancreatic head. The pancreatic duct was not dilated. The gall bladder was not visualized. The stomach and bowel loops looked grossly normal. The spleen and the kidneys were also normal [see [Figure 1] and [Figure 2]. An impression of obstructive jaundice secondary to tumor in pancreatic head was made.
|Figure 2: Showing non-enhanced enterogenous mass in the region of pancreatic head|
Click here to view
The patient had exploratory laparotomy and the following were found: Hepatomegaly, ascites, bile stained organs and the common bile duct and the gall bladder were dilated. A tumor was seen in the distal common bile duct extending to the head of pancreas. Cholecysto-jejunostomy was done. The fine needle aspiration cytology (FNAC) confirmed ductal adenocarcinoma. The patient post-operative condition was satisfactory until 6 th post-operative day when she developed dehisced cholecysto-jejunal anastomosis with leakage of bile from the wound. A 2 nd exploratory laparotomy was carried out to refashion the cholecysto-jejunal anastomosis. On the 2 nd day after the 2 nd operation, the patient developed upper gastrointestinal tract hemorrhage and died on the 9 th day following the 2 nd operation from hypotension and acute renal failure.
| Discussion|| |
Carcinoma of the extra hepatic biliary tree has been recognized for more than a century.  In 1889, Musser reviewed 18 cases of primary extrahepatic bile duct cancer from the literature.  Stewart and associates reviewed 306 cases reported in the world literature up to 1936.  Two decades later, Sako and colleagues reviewed the literature from 1935 to 1954 and found 570 additional cases of extrahepatic bile duct cancer.  In 1957, Altemeire et al,  reported three cases of primary adenocarcinoma of the major intrahepatic bile duct and in 1965; Klatskin described 13 patients with cancers of the hepatic duct bifurcation.  The last report stimulated interest in this uncommon malignancy at the hepatic duct bifurcation often termed a "Klatskin tumor". This case report had the lesion in the extrahepatic. Over the past century, a large amount of information has accumulated concerning the pathology, clinical manifestations and outcome in patients with CC. However, only in the past two decades have improved radiographic techniques allowed prompt and accurate diagnosis at a stage where aggressive therapeutic approaches permit resection or effective palliative measures.  Distal extrahepatic tumors are located in upper border of pancreas to ampulla and more than 95% of these tumors are ductal adenocarcinomas, with many patients presenting with unresectable disease,  as demonstrated in this case report.
The etiology of most bile duct cancers remains undetermined.  Currently, it is believed that gall stones do not increase the risk of CC.  Chronic viral hepatitis and cirrhosis also do not appear to be risk factors.  The identified risk factors include infection such as liver flukes and Ascaris lumbricoides, the latter is common in this environment.  Inflammatory bowel diseases like primary sclerosing cholangitis (PSC) and long-standing ulcerative colitis have a strong relationship with CC.  Other risk factors include chemical exposures primarily among workers on the aircraft, rubber, wood-finishing industries and occasionally CC has developed years after the administration of the radio opaque medium, thorium dioxide (thorotrast).  Congenital diseases of the biliary tree such as choledochal cysts and Caroli disease have also been associated with CC.  None of the risk factors highlighted was established in this case report.
Typically, the diagnosis of obstructive jaundice is suspected on the basis of clinical setting and the results of routine liver function tests.  Diagnostic evaluation follows the standard approach for patient with obstructive jaundice and the initial radiographic studies consist of either an abdominal ultrasound or a CT scan.  If the patient is young and without weight loss or back pain, sonography is favored but, in older patients with weight loss or back pain, a CT scan is preferred, as it may provide substantive evidence to support alternative diagnosis, such as periampullary carcinoma.  Findings at ultrasound or CT suggestive of CC include a dilated intra-hepatic ducts, a normal or collapsed gall bladder and extra-hepatic biliary tree as documented in this case report. But the primary tumor mass is seen in only 20% of patients by ultrasound and in 40% of patients by CT.  Both the ultrasound and CT demonstrated the tumor mass in this report. After ultrasound or CT, documentation of intra-hepatic bile duct dilatation, the biliary anatomy is defined by either percutaneous trans-hepatic cholangiography (PTC) or endoscopic retrograde cholangiopancreatography (ERCP).  The former is favored because it defines the extent of the proximal tumor involvement at the hepatic hilum and permits the preoperative placement of percutaneous trans-hepatic ring catheter. 
The staging of patients with CC usually is accomplished using CT scan, cholangiography and visceral angiography. ,, The uncommon CT findings of bilobar peripheral hepatic metastases or extra-hepatic disease preclude curative resection.  The finding by cholangiography of extensive bilobar hepatic parenchymal involvement indicates unresectability as does the angiographic finding of tumor encasement or occlusion of the common hepatic artery or main portal vein by tumor. , Magnetic resonance imaging holds promise as non-invasive tool for assessing tumor extent and invasion of major portal vascular structures.  On magnetic resonance (MR) images, cholangiocarcinomas appear hypointense on T1-weighted images, and hyperintense on T2-weighted images. Central hypointensity can be seen on T2-weighted images and correspond to fibrosis. On dynamic MR images, cholangiocarcinomas show moderate peripheral enhancement followed by progressive and concentric filling in the tumor with contrast material. Pooling of contrast within the tumor on delayed MR images is suggestive of peripheral cholangiocarcinoma. The role of MR imaging in hilar cholangiocarcinoma is to confirm/reach a diagnosis and to assess respectability.  Hilar cholangiocarcinoma shows the same signal intensity pattern of peripheral tumors both on T1- and T2-weighted images. On magnetic resonance cholangiopancreatography (MRCP) images, hilar cholangiocarcinoma appears as a moderately irregular thickening of the bile duct wall (≥5 mm) with symmetric upstream dilation of the intrahepatic bile ducts.  The aim of preoperative investigation in Klatskin tumors typically requires the evaluation of the level of biliary obstruction, the intrahepatic tumor spread, and the vascular involvement; it also needs to show any atrophy-hypertrophy complex.  Because of its intrinsic high tissue contrast and multiplanar capability, MR imaging and MRCP are able to detect and preoperatively assess patients with cholangiocarcinoma, investigating all involved structures such as bile ducts, vessels and hepatic parenchyma. 
New technique like preliminary evaluation with positron emission tomography (PET) has shown promise in diagnosing the background of primary sclerosing cholangitis and small lesions, measuring less than 1 cm have been demonstrated.  Other new techniques like endoscopic ultrasonography enable bile duct visualization, nodal evaluation and tumor aspiration for cytology.  Intraductal endosonography allows direct ultrasonographic evaluation of the lesion.  Unfortunately, these imaging modalities and techniques are not available in this center.
In conclusion, CC is a rare tumor and the purpose of this case report is to present a case of CC masquerading as tumor of the head of pancreas on ultrasound and CT so as to raise the index of suspicion of the radiologist, that despite the infrequent occurrence of CC, its diagnosis should be considered in every case of obstructive jaundice, and to emphasize that carcinoma of the distal common bile duct presents diagnostic and therapeutic dilemmas.
| References|| |
|1.||Kennedy A, Darwin P. Cholangiocarcinoma. J E Med 2001;2:536-42. |
|2.||Klatskin G. Adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatis: An unusual tumour with distinctive clinical and pathologic features. Am J Med 1965;38:241-5. |
|3.||Sako K, Seitzinger GL, Garside E. Carcinoma of the extrahepatic bile ducts: Review of the literature and report of six cases. Surgery 1957;4:416-20. |
|4.||De Groen PC, Gores GJ, La Russo NF, Gunderson LL, Nagorney DM. Biliary tract cancers. N Engl J Med 1999;341:1368-78. |
|5.||Musser JH. Primary Cancer of the Gall-Bladder and Bile-Ducts. Bost Med Surg J 1889;121:553-7. |
|6.||Stewart HL, Lieber MM, Morgan DR. Carcinoma of the extrahepatic bile ducts. Arch Surg 1940;41:662-713. |
|7.||Altemeier WA, Gall EA, Zinnijger MM, Hoxworth PI. Sclerosing carcinoma of the major intrahepatic bile ducts. AMA Arch Surg 1957;75:450-60. |
|8.||Yeo CJ, Pitt HA, Cameron JL. Cholangiocarcinoma. Surg Clin North Am 1990;70:1429-47. |
|9.||Lillemoe K, Kennedy A, Picus J. Clinical management of carcinoma of the biliary tree. In: Principles and practice of GI. Oncology 2000;1:58-62. |
|10.||Douglas HO, Tepper J, Leichman L. Neoplasm of the extrahepatic bile ducts. Cancer Med 1993;2:1455-62. |
|11.||Chalasani N, Baluyat A, Ismail A, Zaman A, Sood G, Ghalib R, et al. Cholangiocarcinoma in patients with primary sclerosing Cholangistis: A multicenter case-control study. Hepatology 2000;31:7-11. |
|12.||Choi BI, Lee JH, Han MC, Kim SH, Yi JG, Kim CW. Hilar cholangiocarcinoma: Comparative study with sonography and CT. Radiology 1989;172:689-92. |
|13.||Cameron JL, Pitt HA, Zinner MJ, Kaufman SL, Coleman J. Management of proximal cholangiocarcinoma by surgical resection and radiotherapy. Am J Surg 1990;159:91-7. |
|14.||Lynn RB, Wilson JA, Cho KJ. Cholargiocarcinoma: Role of percutaneous transhepatic cholangiography in determination of respectability. Dig Dis Sci 1988;33:587-91. |
|15.||Voyles CR, Bowley NY, Allison DJ, Benjamin S, Blumgart LH. Carcinoma of the proximal extrahepatic biliary tee: Radiographic assessment and the therapeutic alternatives. Ann Surg 1983;197:188-92. |
|16.||Manfredi R, Barbaro B, Masselli G, Vecchioli A, Marano P. Magnetic resonance imaging of cholangiocarcinoma. Semin Liver Dis 2004;24:155-64. |
|17.||Van Beers BE. Diagnosis of cholangiocarcinoma. HPB (Oxford) 2008;10:87-93. |
|18.||Keiding S, Hansen SB, Rasmusen HH, Gee A, Kruse A, Roelsgaard K, et al. Detection of cholangiocarcinoma in Primary sclerosing cholangitis by positron emission tomography. Hepatology 1998;28:700-6. |
[Figure 1], [Figure 2]