DOCTOR'S VIEWS ARCHIVE
Nov. 1, 1999 -- The American football legend Walter Payton died today at his home near Chicago at age 45. Mr. Payton was the all-time leading rusher in the history of the National Football League.
It was well known that he had a progressive liver disease called primary sclerosing cholangitis. However, the cause of Mr. Payton's death was bile duct cancer, said Dr. Greg Gores, his physician at the Mayo Clinic in Rochester, Minnesota.
During a news conference in Chicago on Feb. 2, Mr. Payton had revealed that he had primary sclerosing cholangitis. He said doctors had told him he would need a liver transplant within two years. Only a week later, this time table was revised and he was told that he needed a transplant by the end of 1999.
Dr. Gores indicated that Mr. Payton had received chemotherapy and radiation treatment after the bile duct cancer was diagnosed but that the cancer had progressed to where "transplantation was no longer a viable option."
The purpose of this article is to provide perspectives on both of Mr. Payton's illnesses: bile duct cancer and primary sclerosing cholangitis.
Bile Duct Cancer
Bile duct cancer is a rare disease in which there are malignant cells in the tissues of the extrahepatic bile duct. The bile duct is a tube that connects the liver and the gallbladder to the small intestine. The part of the bile duct that is outside the liver is called the extrahepatic bile duct.
A fluid called bile, which is made by the liver and breaks down fats during digestion, is stored in the gallbladder. When food is being broken down in the intestines, bile is released from the gallbladder through the bile duct to the first part of the small intestine.
If there are symptoms, tests are usually ordered to see if there is cancer. These may include an ultrasound (a test that uses sound waves to find tumors), a CT (a computed tomographic) scan, a special type of x-ray that uses a computer to make a picture of the inside of the abdomen) and an MRI (magnetic resonance imaging which uses magnetic waves to make a picture of the inside of the abdomen).
A test called an ERCP (endoscopic retrograde cholangiopancreatography) may be done. A flexible tube is put down the throat, through the stomach, and into the small intestine. The doctor can see through the tube and inject dye into the drainage tube (duct) of the pancreas so that the area can be seen more clearly on an x-ray.
PTC (percutaneous transhepatic cholangiography) is another test that can help find cancer of the extrahepatic bile duct. During this test, a thin needle is put into the liver through the right side of the patient. Dye is injected through the needle into the bile duct in the liver so that blockages can be seen on x-rays.
If abnormal tissue is found, the doctor may remove a small amount of fluid or tissue from the bile duct and look at it under the microscope to look for cancer cells. This biopsy procedure is usually done during the PTC or ERCP.
Because it is sometimes hard to tell whether a patient has cancer or another disease, surgery may be needed. If this is the case, the surgeon will go into the abdomen and look at the bile duct and the tissues around it for cancer. If there is cancer and if it looks like it has not spread to other tissues, the doctor may remove the cancer or relieve blockages caused by the tumor.
If extrahepatic bile duct cancer is found, further tests are done to see if the cancer cells have spread to other parts of the body. This is called staging the cancer. The stage of the cancer is important to the treatment plan and prognosis (outlook). The following stages are commonly used for extrahepatic bile duct cancer:
- Localized: The cancer is only in the area where it began and it can be removed by surgery.
- Unresectable: The cancer cannot be completely removed (resected) by surgery. The cancer may have spread to nearby organs and lymph nodes or to other parts of the body.
- Recurrent: This means the cancer has come back (recurred) after it has been treated. It may come back in the bile duct or in another part of the body.
The treatments for bile duct cancer include: surgery (taking out the cancer or taking steps to relieve symptoms caused by the cancer), radiation therapy (using high-dose x-rays to kill the cancer cells); and chemotherapy (using drugs to kill cancer cells).
The chances of recovery (prognosis) and the choice of treatment depend on the location of the cancer in the bile duct, the stage of the cancer and the patient's general health.
Primary Sclerosing Cholangitis
This is a chronic disorder of the liver in which the bile ducts outside the liver (the extrahepatic bile ducts) and often the bile ducts inside the liver (the intrahepatic bile ducts) become inflamed, thickened (sclerotic), narrowed, and finally obstructed. This is a progressive process that can in time destroy the bile ducts.
Primary sclerosing cholangitis (PSC) is also commonly called idiopathic sclerosing cholangitis ("idiopathic" means the cause is unknown) or just sclerosing cholangitis. The cause is not known. The disease can occur in isolated form (by itself) or in association with other diseases, including:
- Inflammatory bowel disease, especially with ulcerative colitis;
- Certain uncommon diseases such as multifocal fibrosclerosis syndrome, Riedel's struma, and pseudotumor of the orbit; and
In AIDS, of course, the changes in the biliary tract are not of unknown origin (idiopathic), but are due to infection. The infectious agents include mycoplasma, cytomegalovirus, and others. Changes in the biliary tract are quite common in AIDS and are similar to those seen in PSC.
The disease causes irreversible scarring of the liver (cirrhosis) and liver failure, leading to the consideration of liver transplantation. PSC is, in fact, one of the more common reasons for a liver transplant.
The diagnosis of PSC is confirmed by demonstration of thickened bile ducts using a special radiological test called cholangiography in which dye is injected into the bile ducts and then x-rays are taken of the injected ducts.
The treatment of PSC (short of liver transplantation) includes the drug cholestyramine (QUESTRAN) to diminish itching, antibiotics for infection, and vitamin D and calcium to prevent bone loss (osteoporosis). Sometimes, balloon dilatation (a procedure in which the bile ducts are stretched open) or surgery to bypass an obstructed bile duct are performed.
The expected length of survival depends on the age of the person, their degree of jaundice (based on their blood bilirubin level), how advanced the PSC is by liver biopsy (whether cirrhosis has developed or not), and the size of the spleen (splenomegaly).
The long-term prognosis for PSC is poor. Wwithout a liver transplant, most patients die within 10 years of the time of diagnosis of the disease.