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Cholangiocarcinoma
What are the types of cholangiocarcinoma?
Cholangiocarcinomas arise from the epithelial cells of the bile ducts. The majority of cholangiocarcinomas (>90 percent) are adenocarcinomas, with squamous cell carcinoma being responsible for most of the remaining cases. Distant metastases are distinctly uncommon in cholangiocarcinoma.
Cholangiocarcinoma may arise in the intrahepatic (least common), perihilar, or distal
(extrahepatic) biliary tree. Bile duct tumors that involve the common hepatic duct bifurcation are referred to as Klatskin tumors regardless of whether they arise from the intrahepatic or extrahepatic portion of the biliary tree.
What is the epidemiology of cholangiocarcinoma?
Cholangiocarcinoma generally presents between 50 and 70 years of age. However, patients with primary sclerosing cholangitis (PSC) and those with choledochal cysts present nearly two decades earlier. The incidence of cholangiocarcinoma is slightly higher in men. This probably reflects the higher incidence of PSC in men.
What are the risk factors for cholangiocarcinoma?
What are the clinical features of cholangiocarcinoma?
i. Painless obstructive jaundice, abdominal pain and weight loss.
ii. Cholangiocarcinoma is often associated with intermittent rather than steadily progressive jaundice.
Discuss the diagnosis of cholangiocarcinoma?
Discuss the management of cholangiocarcinoma?
Surgery is the only curative treatment for patients with cholangiocarcinoma.
Criteria for resectability — the traditional guidelines for resectability in US include:
However, as a general rule, true resectability is ultimately determined at surgery, particularly with perihilar tumors.
What is the role of preoperative biliary decompression?
This is controversial. Many surgeons proceed directly to laparotomy without preoperative biliary drainage. On the other hand, there is often uncertainty as to resectability as well as the timetable of surgical evaluation and operative management in patients presenting with jaundice. As a practical issue, stents are often placed to alleviate jaundice while these issues are being settled.
What is the role of chemoradiotherapy?
Neoadjuvant or adjuvant chemotherapy or radiotherapy therapy is not shown to be beneficial in patients with cholangiocarcinoma.
What are the palliative treatment options?
What is the prognosis?
Surgery for cholangiocarcinoma is usually extensive and have a high operative mortality (5%-10%) and low curability. There is a 9–18% five year survival for proximal bile duct lesions and 20–30% for distal lesions.
Median survival for patients with intrahepatic cholangiocarcinoma without hilar involvement is 18–30 months and 12–24 months with perihilar involvement.
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