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Figure 1. Location of the biliary tree in the body.
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Authors: Sanjay B. Jagannath, MD; Charles J. Yeo, MD; Sergey Kantsevoy, MD; Lynn Norwitz, BS; Anthony N. Kalloo, MD
The terms cholangiocarcinoma and bile duct cancer are often used interchangeably. Primary biliary tract malignancies affect one in every 100,000 people per year in the United States. More than 95% of these malignancies are cholangiocarcinomas (epithelial adenocarcinomas ) frequently found in the extrahepatic biliary tree. This form of cancer is slightly more prevalent in males than females (1.3:1.0) and usually affects patients in the fifth to seventh decade of life.
What is Cholangiocarcinoma?
Cholangiocarcinoma is a primary malignant tumor originating from cells that resemble biliary epithelium. The gross appearance is that of one or more firm, white masses (Figure 2).
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Figure 2. A, Extrahepatic tumor; B, intrahepatic tumor resulting in biliary duct dilation. |
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The tumor(s) is usually small and may arise anywhere along the biliary tree, from the small intrahepatic bile ducts to the common bile duct. Microscopically, cholangiocarcinoma may resemble adenocarcinoma. These bile ductule tumors may be well differentiated, while others are poorly differentiated (Figure 3).
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Figure 3. A, Cholangiocarcinoma within a cirrhotic liver; B, cross-section of tumor; C, histological imaging showing tumor cells surrounding normal hepatocytes. |
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Cholangiocarcinomas are usually slow-growing tumors that spread locally via the lymphatic system. Treatment and long-term prognosis are dependent upon the location of the mass. Lesions located in the distal or middle portion of the extrahepatic bile duct (20% and 35%, respectively) have a better prognosis than tumors in the proximal third, which include about 45% of bile duct cancers (including Klatskin’s tumors — hilar variants).
Large solitary tumors are characteristic of peripheral cholangiocarcinoma; however, a multinodular type may occur. These tumors have a fibrous stroma, are firm and grayish white in color, and are not well vascularized. Hilar cholangiocarcinoma are usually firm, intramural, annular tumors that encircle the bile duct, or may be bulky hard masses that are on the duct or hilar region and extend into the liver. They may also appear as a spongy friable mass in the lumen of the bile duct. There may be metastatic nodules throughout the liver with dilation of bile ducts peripheral to the mass.
Symptoms
The clinical presentation of cholangiocarcinoma depends on the anatomic location of the tumor(s). Patients with hilar cholangiocarcinoma, (tumor located in the area of confluence of right and left hepatic ducts) most commonly present with jaundice, pruritis, abdominal pain, fever, weight loss and/or progressive weakness (Figure 4). Patients with peripheral cholangiocarcinoma (tumor originating from small intrahepatic ducts) may present only with vague abdominal pain, unexplained weight loss, weakness and worsening fatigue. Jaundice and pruritus may not be apparent until very late in the disease course, when there is occlusion of segmental bile ducts. Patients with distal cholangiocarcinoma (tumors involving extrahepatic bile ducts) usually have early onset of jaundice and pruritus without abdominal pain. Upon physical examination, these patients usually have a palpable distended gallbladder (Courvoisier’s sign).
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Figure 4. Common symptoms of cholangiocarcinoma. |
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Cholangiocarcinoma may occur in the setting of primary sclerosing cholangitis and may be difficult to diagnose. Clues that may suggest an underlying carcinoma include a stricture that is refractory to therapy, or sudden deterioration in biochemical tests of liver function.
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