Klatskin tumor is one of the bile duct carcinomas. It is considered a special type of cholangiocellular carcinoma.
What is a Klatskin tumor?
Klatskin tumor is a malignant growth that forms on the central bile ducts. It represents a particular variant of bile duct carcinoma. The Klatskin tumor is located at the hepatic fork. At this point, the right and left hepatic ducts join to form a common duct. In medicine, the tumor is also called carcinoma of the hepatic fork or bifurcation carcinoma. People aged 60 to 70 years are particularly affected by Klatskin tumor.
Causes
Klatskin tumor is classified as a cholangiocellular carcinoma, the causes of which are still unclear. For this reason, no precise triggers for the development of this rare disease have yet been found. However, certain risk factors are known to favor the occurrence of a Klatskin tumor. These include pre-existing conditions such as gallstones in the liver (hepatolithiasis), primary sclerosing cholangitis, Caroli syndrome, and a choledochal cyst. Infections with parasites such as Clonorchis sinensis and Opisthorchis viverrini are another risk factor. These diseases occur primarily in China and Thailand. Physicians divide Klatskin tumor into two forms. These are the polypous intraluminal type and the diffuse infiltrating type. Both forms have the possibility of merging into each other. Along the bile ducts, they affect the liver. In about half of all malignancies, metastases to the lymph nodes are also evident at diagnosis. The Klatskin tumor infiltrates the perineural sheaths at an early stage, which may extend to the periortic nerve plexus. Since the spread of the tumor is difficult to detect with imaging examination methods, only histological examination reveals its extent. Klatskin tumor is further subdivided into types I through IV. In type I, the hepatic fork is not affected by the carcinoma, which forms on the bile ducts. On the other hand, if there is involvement of the hepatic fork, we are talking about type II. In the case of type III, the tumor reaches the segmental ducts on one side. If type IV is present, the secondary confluences on the right and left sides are affected. In this case, curative resection can no longer be performed.
Symptoms, complaints, and signs
In most cases, the development of a Klatskin tumor is not even noticed by the patient. Complaints only occur when the tumor increases in size. Then the gallbladder enlarges below the right rib, which is painless. In addition, those affected suffer from severe jaundice (icterus). In most cases, there is also a greater loss of weight. Other complaints may include itching and fatty stools (steatorrhea). In addition, the Klatskin tumor penetrates into the liver. As the disease progresses, there is discomfort in the upper abdomen, loss of appetite and fatigue. Jaundice, which starts at an early stage, is caused by a blockage of the bile ducts. Thus, the outflow of the bile pigment bilirubin is disturbed. The absence of bile acid causes fatty acids and trialcylglycerides to fail to conjugate in the intestine. Moreover, they can no longer be cleaved and absorbed by lipases. Because they remain within the intestinal lumen, the result is fatty stools.
Diagnosis and disease progression
Several examination methods are suitable for diagnosing a Klatskin tumor. These usually involve a blood test for changes in bile and liver enzymes. In addition, abdominal ultrasonography (ultrasound examination of the abdomen) takes place. In an X-ray examination, the patient takes a contrast medium beforehand to make the bile ducts visible. In this way, narrowing of the bile ducts caused by a tumor can be identified. Magnetic resonance cholangiopancreaticography (MRCP) can also be performed to visualize a tumor stenosis. In this case, there is no risk of germ introduction by a contrast agent. However, the Klatskin tumor cannot be visualized in its entire extent by imaging techniques because it spreads along the nerve sheaths. Its core area, however, is usually well detected. A Klatskin tumor often takes a negative course.For example, it has usually already grown to such an extent at the time of diagnosis that surgery can no longer be performed. For this reason, life expectancy with this disease is considered low. Only about five percent of all patients are still alive five years after diagnosis.
Complications
Because Klatskin tumor is a cancer, the usual complications and risks of tumor disease occur in this case. In an unfavorable case or if treatment is initiated late, this tumor can already spread to other regions of the body and thus affect other healthy tissue. This can also lead to a reduced life expectancy. In the worst case, the affected person dies as a result of the tumor. It is not uncommon for the Klatskin tumor to cause jaundice and severe weight loss. In many cases, the affected person also suffers from pain in the abdomen and loss of appetite. Deficiency symptoms and fatty stools continue to occur. The quality of life of the affected person is considerably reduced by the Klatskin tumor and the ability to cope with stress also drops sharply. The tumor can be diagnosed relatively easily, so that treatment can be initiated at an early stage. However, the tumor cannot be successfully treated and removed in every case. The removal is done by surgery and further by radiation. In most cases, the affected person is also dependent on control examinations after the treatment. If necessary, life expectancy may be reduced due to the Klatskin tumor.
When should you see a doctor?
If symptoms of jaundice are noticed, a doctor should be called immediately. Klatskin tumor usually increases in size slowly. Early treatment will prevent permanent damage to the gallbladder and surrounding organs. If other signs of the disease appear, such as noticeable weight loss or problems with bowel movements (for example, fatty stools), medical advice is needed. Individuals who feel tired and fatigued for seemingly no reason should also talk to a doctor. Klatskin tumor occurs in association with gallstones, Caroli syndrome, and primary sclerosing cholangitis, among other conditions. Patients suffering from any of these conditions should consult the appropriate physician. There is also an increased risk of developing a Klatskin tumor after parasitic infections such as Clonorchis sinensi and Opisthorchis viverrini – immediate diagnosis is indicated. At the latest, if pain occurs in other parts of the body, the symptoms must be taken to the family doctor. Other contacts are the internist or a specialist in diseases of the gallbladder.
Treatment and therapy
As part of treatment for Klatskin tumor, an attempt is made to decompress the obstructed bile ducts. The best method for this is endoscopic retrograde cholangiopancreaticography (ERC), in which the patient receives a stent. In this case, transpapillary drainage is preferred over transcutaneous transhepatic drainage. Thus, it is necessary to drain bile to the outside, as it is important in the intestine for digestion. In advance, it may be necessary to create an initial transhepatic access. This is subsequently converted to an internal stent, the outflow of which extends into the duodenum. If surgical removal of the Klatskin tumor is still possible, this is performed. This is followed by chemotherapy. If surgery can no longer be performed, radiation therapy or chemotherapy is given. However, since the diagnosis is usually made at a late stage, in most cases it is no longer possible to achieve tumor freedom. Therefore, symptomatic treatment is usually given, the goal of which is to control jaundice, indigestion and itching.
Outlook and prognosis
The prognosis and outlook of Klatskin tumor is unfortunately very poor. Due to the usually very late diagnosis, only very advanced stages of the disease are detected and accordingly can only be inadequately treated. Because the tumor is then usually already very scattered and thus inoperable, life expectancy is very poor overall, with only a few patients surviving longer than five years after initial diagnosis. If surgery is still possible, it is very radical in order to remove the tumor as completely as possible. Great progress has already been made here in recent years.If this curative and life-prolonging option can no longer be carried out, palliative measures are taken to guarantee the patients a higher quality of life; freedom from pain and activity of the affected persons are in the foreground here. One measure, for example, is splinting the bile ducts to prevent bile stasis and thus liver damage. The patient can also be offered palliative chemotherapy or radiation of the tumor in combination; in the palliative concept, the patient is the focus of attention and can determine which therapies he or she wishes to receive.
Prevention
Because the exact causes of a Klatskin tumor are unknown, no effective preventive measures are available.
Follow-up care
Follow-up care immediately follows the initial treatment of a tumor. Doctors thereby aim to prevent recurrence of symptoms and improve life expectancy by starting treatment early. Klatskin tumor also belongs to the group of these diseases. The decisive factor for the type of aftercare is whether surgery is possible or not. If surgery can no longer be performed, only palliative aftercare takes place. This means that the patient receives long-term treatment until the end of his or her life, during which the symptoms are alleviated as best as possible. If, on the other hand, surgery promises a successful treatment, this leads to close follow-up controls. Depending on the progress of tumor formation, follow-up care may initially be necessary after only six weeks. With increasing freedom from symptoms, the rhythm widens. After the fifth year without new disease, doctors generally recommend follow-up once a year. Follow-up care usually takes place in the hospital where the first operation was performed. The diagnosis is made on the basis of a blood sample. Imaging methods, namely computer tomography, magnetic resonance imaging and endoscopic examinations, are usually used as well. The initial intervention may be followed by rehabilitation. The aim of this is to integrate the patient into everyday life. Psychological and social problems can be addressed. Pain medication can also be adjusted there.
What you can do yourself
Once a Klatskin tumor has been diagnosed, it is important to remove it surgically as soon as possible. Early surgery is necessary to prevent further enlargement of the carcinoma and thus a negative progression. Which measures the patient can still take can only be answered by the responsible physician. In general, the usual preoperative measures should be taken to ensure a complication-free procedure. After the operation, the patient should take it easy and stay in bed. It may be necessary to change the diet, as the gallbladder is weakened by the operation. Accompanying regular control examinations with the physician are indicated. There is always a risk that recurrences may form or that the operation may still cause disorders of the gallbladder months or years later. Therefore, close medical monitoring is the most important self-help measure. If the Klatskin tumor has been discovered too late, therapeutic counseling is advisable. The relatively short life expectancy can cause shock, which is why psychological support, as well as help from friends and family members, is necessary. Symptomatic treatment is still possible and can be supported by the affected person with general measures such as a healthy lifestyle and sparing.