سرطان الأقنية الصفراوية النقيري

سرطان الأقنية الصفراوية النقيري هو أحد أنواع سرطانات القناة الصفراوية والذي يظهر في القنوات الصفراوية التي تؤدي إلى الكبد (القنوات الكبدية) وينضم للمرارة. ويُعرَف أيضًا بسرطان الأقنية الصفراوية النقيري بأورام كلاتسكين.

الأعراض

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.

الأسباب

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.

عوامل الخطورة

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.

التشخيص

من الاختبارات والإجراءات المستخدمة لتشخيص سرطان القنوات الصفراوية، ما يلي:

  • الفحوصات التصويرية، مثل التصوير المقطعي المحوسب والتصوير بالرنين المغناطيسي
  • فحوصات الدم للكشف عن المستويات المرتفعة جدا من مؤشر الورم CA 19-9
  • الخزعة باستخدام تقنية تصوير الأقنية الصفراوية والبنكرياس بالطريق الراجع عبر التنظير الداخلي، أو التصوير بالموجات فوق الصوتية عبر التنظير الداخلي، والفحوصات المخبرية المتخصصة باستخدام التهجين الموضعي المتألق

    لا ينصح الأطباء عادةً بجمع الخلايا باستخدام الإبرة التي تُدخَل عبر الجلد إلى داخل قناة الصفراء (الخزعة عبر الصفاق). حيث تزيد هذه الطريقة خطر تكرر الإصابة بالسرطان وقد تجعل المريض غير مؤهل لعملية زراعة الكبد.

العلاج

سيعتمد اختيار العلاج الأفضل لك على مدى إصابتك بسرطان القنوات الصفراوية النقيري وموقعها. عادةً ما يتضمن العلاج إجراء جراحة أو زراعة كبد أو علاجات داعمة لتقليل الألم والأعراض الأخرى.

الجراحة

تشمل جراحة سرطان الأقنية الصفراوية الهلالي استئصال ما يلي:

  • الخلايا السرطانية
  • القنوات المرارية المحيطة
  • أجزاء من الكبد
  • العُقَد اللمفية المجاورة

يوصّل الجرّاح بعد ذلك القنوات المرارية المتبقية بالأمعاء الدقيقة حتى يظل بإمكان المرارة الوصول إلى السبيل الهضمي للمساعدة على هضم الطعام.

زراعة الكبد

يمكن لبعض المصابين بسرطان الأقنية الصفراوية النقيري في مراحله المبكرة التفكير في إجراء زراعة الكبد. عادةً ما يُستخدم العلاج الكيميائي والإشعاعي قبل زراعة الكبد لتدمير أكبر قدر ممكن من الخلايا السرطانية قبل الجراحة.

وتتضمن خطة العلاج هذه عادةً ما يلي:

  • علاج كيميائي يُعطى من خلال الوريد مع دواء يُضعف مقاومة الخلايا للإشعاع
  • علاج بالحزم الإشعاعية الخارجية
  • علاج بالإشعاع الداخلي (معالجة كَثَبِيّة) باستخدام أسلاك صغيرة تُوضع بالقرب من موضع السرطان ويُطلق عبرها الإشعاع ببطء
  • علاج كيميائي على هيئة أقراص لحين إجراء عملية الزراعة
  • إجراء جراحة طفيفة التوغل للكشف عن مؤشرات انتشار السرطان
  • زراعة الكبد إذا توفر كبد من متبرع

قد تُجرى لك اختبارات روتينية لتقييم حالتك الصحية أثناء انتظار عملية زراعة الكبد، للتأكد من أنك بصحة جيدة بما يكفي لإجراء الجراحة.

العلاج الكيميائي

يمكن لمرضى سرطان الأقنية الصفراوية النقيري المتقدم الذين لا يمكنهم الخضوع للجراحة أو زراعة الكبد أن يتلقوا العلاج الكيميائي لإبطاء نمو السرطان.

التأقلم والدعم

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?
06/06/2023

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سرطان القنوات الصفراوية (سرطان الأقنية الصفراوية)