肝门部胆管癌
Cholangiocarcinoma
Cholangiocarcinoma
Cholangiocarcinoma, also called bile duct cancer, happens in the tubes that carry the digestive fluid bile from the liver to the small intestine. The tubes are called bile ducts. When bile duct cancer forms in the ducts within the liver it's called intrahepatic cholangiocarcinoma. Bile duct cancer that happens in the ducts just outside the liver is called hilar cholangiocarcinoma. Bile duct cancer that happens near the small intestine is called distal cholangiocarcinoma.
肝门部胆管癌是一种癌症，见于直通肝脏（肝管）和连接胆囊的胆管。肝门部胆管癌也称为 Klatskin 瘤。
症状
Hilar cholangiocarcinoma may not cause symptoms at first. Symptoms typically appear when the cancer grows and blocks the bile ducts. Signs and symptoms of hilar cholangiocarcinoma may include:
- Yellowing of the skin and the whites of the eyes, known as jaundice.
- Itching.
- Dark urine.
- Clay-colored stools.
- Stomach pain.
- Fatigue.
- Weight loss.
When to see a doctor
Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.
病因
胆囊和胆管
胆囊和胆管
胆囊储存肝脏产生的一种黄绿色液体，称为胆汁。胆汁从肝脏流入胆囊，并储存在胆囊中，直到需要用来帮助消化食物。进食过程中，胆囊会将胆汁释放到胆管中。然后胆管将胆汁带到小肠上部（称为十二指肠），以帮助分解食物中的脂肪。
It's not clear what causes most hilar cholangiocarcinomas. This cancer starts in the bile ducts that connect the liver to the small intestine.
Hilar cholangiocarcinoma happens when cells in a bile duct develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to grow and multiply quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells.
The cancer cells form a mass called a tumor. The tumor can grow to invade and destroy healthy body tissue. In time, cancer cells can break away and spread to other parts of the body. When cancer spreads, it's called metastatic cancer.
风险因素
Factors that may increase the risk of hilar cholangiocarcinoma include:
- Primary sclerosing cholangitis. Primary sclerosing cholangitis causes hardening and scarring of the bile ducts that can lead to cancer.
- Older age. Hilar cholangiocarcinoma happens most often in adults over age 50.
- Inflammatory bowel disease. People who have ongoing inflammation of the digestive tract have an increased risk of hilar cholangiocarcinoma.
- Liver cirrhosis. Liver cirrhosis causes scarring of the liver that increases the risk of hilar cholangiocarcinoma.
- Bile duct cysts. Bile duct cysts cause dilated and irregular bile ducts. Having bile duct cysts increases risk of hilar cholangiocarcinoma.
- Gallbladder conditions. Ongoing inflammation of the gallbladder, called cholecystitis, increases the risk of hilar cholangiocarcinoma. People who develop gallstones also are at an increased risk.
- Liver parasites. In areas of Southeast Asia, hilar cholangiocarcinoma is linked to liver flukes, which are parasites that can infect humans. Liver fluke infection can happen from eating raw or undercooked fish.
诊断
用于诊断肝门部胆管癌的检查和医疗程序包括：
治疗
哪种治疗最适合您取决于肝门部胆管癌的位置和累及范围。治疗方案通常包括手术、肝移植或用以减轻疼痛和其他症状的辅助性治疗。
外科手术
肝门部胆管癌的手术涉及切除以下组织：
然后外科医生会将剩余胆管接到小肠上，这样胆汁就仍然能到达消化道，并帮助消化食物。
肝脏移植
某些早期肝门部胆管癌患者可考虑肝移植。肝移植术前通常进行化疗及放射治疗以在术前尽可能多的杀死肿瘤细胞。
治疗通常包括：
- 静脉注射化疗药物使您的细胞更容易接受辐射
- 外照射疗法
- 内放射（近距离治疗）通过在肿瘤附近放置小导线缓慢释放辐射
- 移植手术前服用化疗药物
- 通过微创手术治疗寻找癌症扩散的迹象
- 当有供肝时进行肝移植
在等待肝移植期间，您可能会接受常规检查来评估健康状况，以确保您的健康状况可接受手术。
化疗
不能接受手术或肝移植的晚期肝门部胆管癌患者可以通过化疗减缓癌症的生长。
妥善处理与支持
With time, you'll find ways to cope with feelings that happen after a cancer diagnosis. Until then, here are some ideas for coping with hilar cholangiocarcinoma.
Learn enough about cancer to make decisions about your care
Ask your healthcare team about your cancer, including your test results, treatment options and, if you want, your prognosis. As you learn more about hilar cholangiocarcinoma, you may become more confident in making treatment decisions.
Keep friends and family close
Keeping your close relationships strong can help you deal with hilar cholangiocarcinoma. Friends and family can provide the practical support you may need, such as helping take care of your home if you're in the hospital. And they can serve as emotional support when you feel overwhelmed by having cancer.
Find someone to talk with
Find someone who is willing to listen to you talk about your hopes and worries. This may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.
Ask your healthcare team about support groups in your area. Other sources of information include the U.S. National Cancer Institute and the American Cancer Society.
Preparing for your appointment
Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.
If your healthcare professional thinks you might have hilar cholangiocarcinoma, you may be referred to a doctor who specializes in diseases of the digestive system, called a gastroenterologist. If a cancer diagnosis is made, you also may be referred to a doctor who specializes in treating cancer, called an oncologist.
Because appointments can be brief, it's a good idea to be prepared. Here's some information to help you get ready.
What you can do
- Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
- Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
- Write down important personal information, including major stresses or recent life changes.
- Make a list of all medicines, vitamins and supplements you're taking and the doses.
- Take a family member or friend along. Sometimes it can be hard to remember all the information provided during an appointment. Someone who goes with you may remember something that you missed or forgot.
- Write down questions to ask your healthcare team.
Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For hilar cholangiocarcinoma, some basic questions to ask include:
- Do I have hilar cholangiocarcinoma?
- What is the stage of my hilar cholangiocarcinoma?
- Has my hilar cholangiocarcinoma spread to other parts of my body?
- Will I need more tests?
- What are the treatment options?
- How much does each treatment increase my chances of a cure or prolong my life?
- What are the potential side effects of each treatment?
- How will each treatment affect my daily life?
- Is there one treatment option you believe is the best?
- What would you recommend to a friend or family member in my situation?
- Should I see a specialist?
- Are there any brochures or other printed material that I can take with me? What websites do you recommend?
- What will determine whether I should plan for a follow-up visit?
Don't hesitate to ask other questions.
What to expect from your doctor
Be prepared to answer questions, such as:
- When did your symptoms begin?
- Have your symptoms been continuous or occasional?
- How severe are your symptoms?
- What, if anything, seems to improve your symptoms?
- What, if anything, appears to worsen your symptoms?