Cholangiocarcinoma

Accessory digestive system.
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Alternative Names: Bile duct cancer

Definition: Cholangiocarcinoma is a cancerous (malignant) growth in the bile duct which drain bile from the liver into the small intestine. Other biliary tract cancers include pancreatic cancer, gall bladder cancer, and cancer of the ampulla of Vater. Cholangiocarcinoma is a relatively rare adenocarcinoma, with an annual incidence of 1–2 cases per 100,000 in the Western world, but rates of cholangiocarcinoma have been rising worldwide over the past several decades.

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Causes
Cancerous tumors of the bile ducts are usually slow-growing and do not spread (metastasize) quickly. However, many of these tumors are already advanced by the time they are found.

A cholangiocarcinoma may start anywhere along the bile ducts. These tumors block off the bile ducts.

They affect both men and women. Most patients are older than 65.

Risk Factors:
Although most patients present without any known risk factors evident, a number of risk factors for the development of cholangiocarcinoma have been described; in the Western world, the most common of these is primary sclerosing cholangitis (PSC), an inflammatory disease of the bile ducts which is in turn closely associated with ulcerative colitis (UC). Epidemiologic studies have suggested that the lifetime risk of developing cholangiocarcinoma for a person with PSC is 10%–15%,  although autopsy series have found rates as high as 30% in this population. The mechanism by which PSC increases the risk of cholangiocarcinoma is not well-understood.
Certain parasitic liver diseases may be risk factors as well. Colonization with the liver flukes Opisthorchis viverrini (found in Thailand, Laos, and Malaysia) or Clonorchis sinensis (found in Japan, Korea, and Vietnam) has been associated with the development of cholangiocarcinoma. Patients with chronic liver disease, whether in the form of viral hepatitis (e.g. hepatitis B or C), alcoholic liver disease, or cirrhosis from other causes, are at increased risk of cholangiocarcinoma. HIV infection was also identified in one study as a potential risk factor for cholangiocarcinoma, although it was unclear whether HIV itself or correlated factors (e.g. hepatitis C infection) were responsible for the association.

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Congenital liver abnormalities, such as Caroli’s syndrome or choledochal cysts, have been associated with an approximately 15% lifetime risk of developing cholangiocarcinoma. The rare inherited disorders Lynch syndrome II and biliary papillomatosis are associated with cholangiocarcinoma. The presence of gallstones (cholelithiasis) is not clearly associated with cholangiocarcinoma. However, intrahepatic stones (so-called hepatolithiasis), which are rare in the West but common in parts of Asia, have been strongly associated with cholangiocarcinoma. Exposure to Thorotrast, a form of thorium dioxide which was used as a radiologic contrast medium, has been linked to the development of cholangiocarcinoma as late as 30–40 years after exposure; Thorotrast was banned in the United States in the 1950s due to its carcinogenicity.

Ricks for this condition include:

* Bile duct (choledochal) cysts
* Chronic biliary irritation
* History of infection with the parasitic worm, liver flukes
* Primary sclerosing cholangitis

Cholangiocarcinoma is rare. It occurs in approximately 2 out of 100,000 people.

Symptoms
* Chills
* Clay-colored stools
* Fever
* Itching
* Loss of appetite
* Pain in the upper right abdomen that may radiate to the back
* Weight loss
* Yellowing of the skin (jaundice)

The most common physical indications of cholangiocarcinoma are abnormal liver function tests, jaundice (yellowing of the eyes and skin), which occurs only when bile ducts are blocked by the tumor, abdominal pain (30%–50%), generalized itching (66%), weight loss (30%–50%), fever (up to 20%), or changes in stool or urine color.To some extent, the symptoms depend upon the location of the tumor: Patients with cholangiocarcinoma in the extrahepatic bile ducts (outside the liver) are more likely to have jaundice, while those with tumors of the bile ducts within the liver often have pain without jaundice.
.Yellowing of the skin and eyes (jaundice)->     CLICK & SEE
Blood tests of liver function in patients with cholangiocarcinoma often reveal a so-called “obstructive picture,” with elevated bilirubin, alkaline phosphatase, and gamma glutamyl transferase levels, and relatively normal transaminase levels. Such laboratory findings suggest obstruction of the bile ducts, rather than inflammation or infection of the liver, as the primary cause of the jaundice.  CA19-9 is elevated in most cases

Diagnosis:–
Cholangiocarcinoma is definitively diagnosed from tissue, i.e. it is proven by biopsy or examination of the tissue excised at surgery. It may be suspected in a patient with obstructive jaundice. Considering it as the working-diagnosis may be challenging in patients with primary sclerosing cholangitis (PSC); such patients are at high risk of developing cholangiocarcinoma, but the symptoms may be difficult to distinguish from those of PSC. Furthermore, in patients with PSC, such diagnostic clues as a visible mass on imaging or biliary ductal dilatation may not be evident.

Exams and Tests:-
Blood tests
Blood tests that show abnormal function.
There are no specific blood tests that can diagnose cholangiocarcinoma by themselves. Serum levels of carcinoembryonic antigen (CEA) and CA19-9 are often elevated, but are not sensitive or specific enough to be used as a general screening tool. However, they may be useful in conjunction with imaging methods in supporting a suspected diagnosis of cholangiocarcinoma.
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Abdominal imaging
CT scan showing cholangiocarcinomaUltrasound of the liver and biliary tree is often used as the initial imaging modality in patients with suspected obstructive jaundice. Ultrasound can identify obstruction and ductal dilatation and, in some cases, may be sufficient to diagnose cholangiocarcinoma.  Computed tomography (CT) scanning may also play an important role in the diagnosis of cholangiocarcinoma.

Tests that show a tumor or blockage in the bile duct:
*Abdominal CT scan
*Abdominal ultrasound
*CT scan-directed biopsy
*Cytology
*Endoscopic retrograde cholangiopancreatography (ERCP)
*Percutaneous transhepatic cholangiogram (PTCA)

Liver function tests (especially bilirubin)

Treatment  :-
The goal is to treat the cancer and the blockage it causes. When possible, surgery to remove the tumor is the treatment of choice and may result in a cure. However, often the cancer has already spread by the time it is diagnosed.

Chemotherapy or radiation may be given after surgery to decrease the risk of the cancer returning. However, the benefit of this treatment is not certain.

Endoscopic therapy or surgery can clear blockages in the biliary ducts and relieve jaundice in patients when the tumor cannot be removed.

For patients with cancer that cannot be removed, radiation therapy may be beneficial. Chemotherapy may be added to radiation therapy or used when the tumor has spread. However, this is rarely effective.

Support Groups:-
You can ease the stress of illness by joining a support group with members who share common experiences and problems (see cancer – support group).

Hospice is often a good resource for patients with cholangiocarcinoma that cannot be cured.

Prognosis:

Surgical resection offers the only potential chance of cure in cholangiocarcinoma. For non-resectable cases, the 5-year survival rate is 0% where the disease is inoperable because distal lymph nodes show metastases[63], and less than 5% in general. Overall median duration of survival is less than 6 months in inoperable, untreated, otherwise healthy patients with tumors involving the liver by way of the intrahepatic bile ducts and hepatic portal vein.

For surgical cases, the odds of cure vary depending on the tumor location and whether the tumor can be completely, or only partially, removed. Distal cholangiocarcinomas (those arising from the common bile duct) are generally treated surgically with a Whipple procedure; long-term survival rates range from 15%–25%, although one series reported a five year survival of 54% for patients with no involvement of the lymph nodes. Intrahepatic cholangiocarcinomas (those arising from the bile ducts within the liver) are usually treated with partial hepatectomy. Various series have reported survival estimates after surgery ranging from 22%–66%; the outcome may depend on involvement of lymph nodes and completeness of the surgery. Perihilar cholangiocarcinomas (those occurring near where the bile ducts exit the liver) are least likely to be operable. When surgery is possible, they are generally treated with an aggressive approach often including removal of the gallbladder and potentially part of the liver. In patients with operable perihilar tumors, reported 5-year survival rates range from 20%–50%.

The prognosis may be worse for patients with primary sclerosing cholangitis who develop cholangiocarcinoma, likely because the cancer is not detected until it is advanced. Some evidence suggests that outcomes may be improving with more aggressive surgical approaches and adjuvant therapy.

Possible Complications :-
*Infection
*Liver failure
*Spread (metastasis) of tumor to other organs.

When to Contact a Medical Professional :-
Call your health care provider if you have jaundice or other symptoms of cholangiocarcinoma.

Disclaimer: This information is not meant to be a substitute for professional medical advise or help. It is always best to consult with a Physician about serious health concerns. This information is in no way intended to diagnose or prescribe remedies.This is purely for educational purpose.

Resources:
http://en.wikipedia.org/wiki/Cholangiocarcinoma
http://www.nlm.nih.gov/MEDLINEPLUS/ency/article/000291.htm

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