Cholangioma

Cholangioma means the cancer generated on the cell surrounding the inside of the bile duct (epithelial cell). The bile duct is classified into the intrahepatic bile duct and the extrahepatic bile duct, and so cholangioma is called ‘intrahepatic bile duct cancer’ and ‘extrahepatic bile duct cancer’ respectively. As the gallbladder is a part of the biliary system, gallbladder cancer and cholangioma are of the same kind in a broad sense. However, gallbladder cancer is ordinarily regarded as different due to histological differences. Intrahepatic bile duct cancer is ordinarily regarded in the same light as hepatoma because it is also generated on the intrahepatic bile duct, but it should be differentiated from hepatoma because two cancers are remarkably different from each other in characteristics and therapeutic methods. The liver is constituted by various cells, and the cancer generated on the hepatocyte is called ‘hepatoma’ and the cancer generated on the intrahepatic bile duct is called ‘intrahepatic bile duct cancer’. Also, two cancers are strikingly different from each other in the causal cell. Cholangioma is mostly characterized by jaundice. In the case of serious jaundice, feces become white and the skin becomes itchy as well as right epigastric pains, pyrexia, weight loss, anorexia, nausea and emesis may be arisen. In the case of cholangioma, blood bilirubin and alkaline phosphatase are increased as well as CA19-901, the tumor marker, is increased by 55 to 65 percent. In the case of radiotherapies, ultrasonography, CT scan, magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) are mostly used. Therapeutic methods are largely divided into two, that is, a method to excise or decrease carcinoma, such as a surgical operation, cancer chemotherapy and radiotherapy, and another method to open the obstructed bile duct. Among such methods, only surgical excision is expected to make patients survive for a long time but the problem is that many patients miss the opportunity to undergo surgical operations. In case the bile duct is obstructed by cholangioma, bile cannot run to the duodenum and so jaundice is arisen. The same as standing water rots, standing bile causes cholangitis and eventually sepsis is arisen by disease germs running through blood vessels. The sepsis patients mostly die, and sepsis accounts for the highest rate of the death of cholangioma patients. Surgical operations, drug treatment or radiotherapy all can open the obstructed bile duct, but such methods cannot be applied to the patients who have serious jaundice due to the risk of cholangitis. In the case of the jaundice caused by cholangioma, the obstructed bile duct should be opened emergently. Other treatments depend on the failure or success of the surgery.

Definition

The bile duct is to run off bile from the liver to the duodenum. Bile is produced by hepatocytes (the cells that constitute the liver) and is secreted into a very narrow tube (canaliculi), and it becomes gradually thicker the same as branches gather toward the trunk. It comes out of the liver, and discharges bile through the papillary part of the duodenum. As foresaid, the bile duct is classified into the intrahepatic bile duct and the extrahepatic bile duct that is stretches into the duodenum. The extrahepatic bile duct, which is about 8cm long, is classified into the hepatic portal part, the upper part, the middle part and the lower part. The cancer generated on such parts is called ‘cholangioma’ and it is a malignant tumor generated on the epithelial cell of the gallbladder. Cholangioma is classified into intrahepatic bile duct cancer and extrahepatic bile duct cancer according to region. In some cases the intrahepatic bile duct cancer generated in the canaliculi is regarded as hepatoma, but it is remarkably different from hepatoma.

Symptoms

In case the bile duct is obstructed by cancer, bile cannot run and the bile duct above the obstructed duct is filled with bile and so pressure is heightened. Eventually, bile flows backward into blood vessels. In such a case, jaundice is arisen and the skin and eyes turn yellow due to the pigment called ‘bilirubin’. Jaundice is divided into two kinds; a jaundice arisen due to the obstructed bile duct, the same as cholangioma and pancreatic caner, and another jaundice arisen due to hepatitis or a certain medicine. The first jaundice is medically called ‘obstructive jaundice’, and the second is arisen as hepatocytes are damaged by hepatitis viruses or drugs. In the case of obstructive jaundice, bile cannot run into the duodenum and so feces turns white due to the pigment ‘bilirubin’ as well as urine turns red and dark as the concentration of bilirubin is heightened in blood. In the case of serious jaundice, pruritus may be arisen because the bile acid in bile is absorbed into blood and deposited under the skin concurrently with bilirubin. In addition, weight loss, anorexia, nausea and emesis may be arisen.

Cause, conditions and physiological

The cause of cholangioma is not clarified as yet, but it is presumed that cholangioma is caused by various conditions that are likely to inflame the cells surrounding the inside of the bile duct. Also, biliary calculus is regarded as a cause by reason it is detected in 20 to 30 percent of cholangioma patients. In addition, congenital disorders such as liver fluke, inflammatory colon diseases and biliary cyst are presumed to be related with cholangioma.

Diagnosis

Cholangioma is characterized by blood bilirubin and the enzyme ‘alkaline phosphatase’ that are increased after onset. The tumor marker is not secreted in normal tissues but is secreted after the onset of cancer, and various tumor markers had been found out in various carcinomas. As it can be simply used with blood test, it has been widely used. Nevertheless, there is no the tumor marker that is of help to diagnose cholangioma directly, as yet. Some tumor markers have been actually used in about 50 percent of cholangioma patients, but it is difficult to entirely depend on such tumor markers because they are not increased in early cancer as well as some advanced cancers do not show increase. As cholangioma is stealthily metastasized into surrounding tissues and conspicuous mass is not shown in many cases, it is not easy to correctly diagnose cholangioma. However, the advance of image analysis is of help to diagnose cholangioma. Radiotherapies are divided into non-invasive methods, such as ultrasonography, CT scan and MRCP, and invasive methods such as ERCP and PTC. Ultrasonography does not give pain to patients, and does not expose them to radial rays, and can be repetitively performed with ease. In addition, ultrasonography is suitable to monitor the bile duct, and is of help to calculate the obstructed par. For these reasons, ultrasonography is preferentially performed. Although ultrasonography is of help to track down cholangioma, it should be examined more correctly in order that the progress of cancer may be clarified. CT scan or MRI scan is typical examination that is performed to analyze the progress of cancer and distant metastasis. ERCP (endoscopic retrograde cholangiopancreatography) is to examine the shape of the bile duct or the pancreatic duct. To perform this method, an endoscope is inserted into the duodenum and a thin tube is put into the papillary part of the duodenum and a contrast medium is injected. Through this method, the obstructed bile duct can be opened as well as biopsy can be performed. ERCP can diagnose the jaundice caused by cholangioma, and can basically treat it. PTC is to contrast the bile duct by penetrating a needle through the skin and the liver. As the stricture and obstruction of the bile duct can be monitored in detail, the position and progress of carcinoma can be analyzed. In case the bile duct is obstructed, a serious complication may be arisen. In such a case, bile can be drained by the drainage tube.

Progress, prognosis

Cholangioma is one of the cancers of which prognoses are not satisfactory. Of course it can be completely recovered by surgical operations in case it is early detected, but patients mostly miss the opportunity to undergo surgical operations. 25 percent of cholangioma patients can undergo surgical operations, and five-year survival rate reaches just 5 percent. Nevertheless, the foregoing result is nothing but a numerical value. As the response of patients is different from each other, therapeutic methods are difficult to be applied to patients in the same light.

Complication

In case obstructive jaundice is arisen by cholangioma, secondarily cholangitis and sepsis are arisen and eventually the patient meets its death. Cholangitis and sepsis, caused by cholangioma, are complications needing emergency treatment. In case cholangioma is metastasized into other organs, various complications are arisen.

Treatment

The therapeutic methods to cholangioma become different as to progress, the same as other cancers. Cholangioma is classified into primary, secondary, tertiary and terminal stage according to progress. The progress of cancer is graded by infiltration, the metastasis into lymph nodes and distantmetastasis. During the primary stage, cancer is limited to the bile duct and the muscle layer. During the secondary stage, cancer is developed more than the primary stage but is not metastasized into adjacent organs and adjacent lymph nodes. During the tertiary stage, cancer is metastasized into other organs but is metastasized into lymph nodes. During the terminal stage, cancer is directly metastasized into other organs including the liver and the peritoneum. Specifically, stages are classified as to the progress of metastasis. Surgical operations, cancer chemotherapy and radiotherapy are typical therapeutic methods that directly remedy cancer, and there is a subsidiary remedy that alleviates the pain. Among such methods, surgical excision is expected to make long-term survival possible. Despite criticism, radiotherapy is of help to alleviate jaundice or the pains after excision. Cancer chemotherapy is not effective to remedy cholangioma, so it should be reviewed with time. It has been recently reported that new anticancer medicines have been developed and such medicines have come to good. The cancer in the primary or secondary stage should be surgically excised. It is desirable to surgically excise the cancer in the tertiary stage if possible, but it is advisable to simultaneously use surgical therapy, radiotherapy and anticancer medicine in case it is widely metastasized. In the case of the terminal stage, in many cases it aims ate relieving symptoms through chemotherapy or radiotherapy. In case obstructive jaundice is arisen by cholangioma, the bile duct should be opened in order that bile may be run down. The bile duct can be linked to the liver by a surgical operation, but recently bile was impossible to be drained by an endoscopic technique or radiological therapy. To perform endoscopic drainage, an endoscope should be inserted into the duodenum and a plastic or metal tube should be inserted from the ampulla of vater into the bile duct. It needs hi-tech skills, but it the obstructed duct can be effectively opened within 20 to 30 minutes. In case endoscopic drainage is difficult to be performed, a drainage tube should be selectively inserted into the bile duct through the skin and the liver.

Preventions

There is no method to fundamentally preventive cholangioma.

Consult a doctor in the following cases

In the following cases, you should immediately go to the doctor.
- A case where a mass is felt at the right abdomen or where a dull pain is repetitively felt
- A case where the eye or the skin turns yellow and where urine turns brownish
- A case where pruritus is arisen concurrently with jaundice
- A case where weight is lost
- A case where fever is developed concurrently with the right epigastric pain

from: http://www.hidoc.co.kr/