Each year approximately 15,000 new cases of liver and biliary tract cancers are diagnosed in the United States. Of those cases, roughly two-thirds are located somewhere in the bile duct system, with an additional 10% containing Klatskin tumors, a serious and rare type of tumor named after Dr. Gerald Klatskin in 1965.
Cancer of the bile ducts can occur anywhere along the course of the biliary system, starting at the liver all moving all the way through to the small intestine. However cancer comprised of Klatskin tumors are located in the upper part of the bile duct system affecting either the right or left hepatic ducts, and sometimes both. Unfortunately, the hepatic ducts are closely associated with the blood vessels that supply blood to the liver so if infected with Klatskin tumors, metastasizing into the liver is not uncommon.
Klatskin Tumor Causes and Symptoms
While there is no official known cause for Klatskin tumors in the bile duct, there are some risk factors that have been associated with a small percentage of those diagnosed because they are known to cause biliary inflammation and may be related to Klatskin tumor formation.
- Primary sclerosing cholangitis
- Secondary sclerosing cholangitis
- Chronic typhoid carriage
- Parasitic infections
- Exposure to thorotrast (an x-ray contrast medium)
- Choledochal Cysts
- Ulcerative colitis
- Crohn’s disease
Most patients are unaware that they have Klatskin tumors as this disease takes a long time to form and rarely shows symptoms due to its location deep within the abdominal cavity.
When a Klatskin tumor is blocking the bile duct and preventing it from draining properly, people will then begin to develop symptoms related to that obstruction.
Symptoms typically include jaundice (extreme yellowing of the skin), clay-colored stools (due to lack of bile drainage into the digestive system), bilirubinuria (dark urine), itchy skin, weight loss, and abdominal pain. Jaundice is usually the symptom prompting a person to seek medical attention.
The primary goal of treatment and therapy for Klatskin cancer is the removal of the diseased tissue and relief of biliary blockages due to tumor formations, though Klatskin tumors are rare and have limited treatment options.
Before any treatment option is decided upon, it is best you speak with a specialized Klatskin cancer physician to determine the extent and severity of your cancer diagnosis.
If your physician suspects you have Klatskin cancer, you may undergo an abdominal ultrasound, a computed tomography (CT) scan, or a magnetic resonance cholangiopancreatography (MRCP) to make a proper diagnosis.
If your physician detects Klatskin tumors after conducting initial screenings of your bile duct system, you may then undergo cholangiography (x-rays of the bile ducts) to pinpoint the severity and location of the tumor growth. You may also receive stents within the biliary system to allow for proper drainage of bile to decrease the presence of jaundice until further treatment is determined.
Surgical Removal of Klatskin Tumors
Surgical resection of the Klatskin tumors is the only possible curative treatment option for Klatskin cancer.
Klatskin tumors usually affect not only the upper bile ducts but the liver as well since they are so close in proximity. Luckily, since the liver is made up of two separate lobes, the right and left, one of the lobes can safely be removed during surgery if the Klatskin tumors have affected only one side of the bile ducts and/or lobes.
Sometimes however, it is difficult to determine whether the cancer has metastasized further into the liver via the blood vessels and only upon exploratory surgery can complete resection decisions be made. That is why it is so important that you entrust only the most specialized Klatskin cancer surgeon to conduct your Klatskin tumor surgery.
Often Klatskin tumors affect both lobes of the liver, the blood vessels supplying the liver, or both the right and left hepatic ducts making curative resection almost impossible.
When a patient with Klaskin tumors is not a good candidate for standard surgical resection, liver transplantation then becomes an option, albeit and long and complicated one at best.
Palliative treatment is designed to relieve symptoms of an illness and improve a person’s quality life. Used during any stage of an illness, palliative treatment comes in many forms.
In the case of Klatskin tumors, it can be used to help ease pain or sickness from either the cancer itself, medicinal treatments the patient may be receiving, or in more advanced stages of cancer, it can be used simply to allow you to live a longer and more comfortable life, especially if the cancer is incurable.
Here are some of the most common forms of palliative care someone with Klatskin tumors may receive from their physician as part of the treatment process:
- Chemotherapy – In its most traditional sense, chemotherapy is usually used as a way to eliminate all traces of cancer with the hope it will never return. However, with cancers that have metastasized beyond a cure, palliative chemotherapy can be used to decrease tumor sizes and decrease the effects the cancer is having on the patient so the patient not only feels better but lives longer as well.
- Radiation – Similar to chemotherapy, palliative radiation is especially useful in alleviating pain that is associated with cancer. Though it may not lengthen a patient’s life or cure the patient of cancer, their quality of life will be much improved.
- Hormone Therapy – This palliative treatment is designed to stunt hormones that may have an effect on the growth of particular tumors a patient has.
- Biological Therapy – This form of palliative care is relatively new when being used for cancer treatment. Using natural or artificial substances that mimic or block natural cell responses, biological therapy attempts to kill, control, or change the behavior of cancer cells in hopes of at least reducing their size and effect on the patient.
- Radiofrequency Ablation – Another great technique for alleviating pain associated with cancer, radiofrequency ablation is a minimally invasive and therapeutic treatment option that uses heat made by radio waves to kill cancer cells.
- Cryotherapy – Using extreme cold to target cancer cells, cryotherapy offers some relief. After the procedure, the patient’s immune system effectively rids itself of the dead tissue that results from the cell freezing.
In patients with advanced metastatic Klatskin cancer, sometimes the only relief that can be offered is from the jaundice symptoms. This is typically done by inserting wall stents into the biliary system to allow for proper drainage of bile during the digestive process. Unfortunately, the stents are prone to obstructions and must be replaced approximately every three months to provide proper relief.
In the end, Klatskin cancer is a disease that must be taken seriously. With a poor prognosis for anyone diagnosed, it is essential that you entrust only the most specialized physician to restore your health.
If you or someone you know has been diagnosed with Klatskin tumors of the upper bile duct system, consider contacting Dr. Fraiman to help you determine your next steps in treatment.
Practicing for over 20 years and dealing with the most complex of liver diseases, Dr. Fraiman and his multi-disciplinary team have a patient-oriented approach to taking care of their patients. With Dr. Fraiman you can rest assured that you will be given the best care possible and that your health will be a priority.