Colangiocarcinoma hiliar
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.
El colangiocarcinoma hiliar es un tipo de cáncer de vías biliares que aparece en las vías biliares que salen del hígado (conductos hepáticos) y se unen con la vesícula biliar. El colangiocarcinoma hiliar también se conoce como tumor de Klatskin.
Síntomas
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.
Causas
Vesícula biliar y conducto biliar
Vesícula biliar y conducto biliar
La vesícula biliar retiene bilis, un líquido amarillo verdoso fabricado por el hígado. La bilis fluye del hígado a la vesícula biliar. Se reserva en la vesícula biliar hasta que sea necesario para digerir alimentos. Cuando comes, la vesícula biliar libera bilis hacia el conducto biliar. El conducto trasporta la bilis hasta el duodeno, la parte superior del intestino delgado, para ayudar a descomponer las grasas de la comida.
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.
Factores de riesgo
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.
Diagnóstico
Las pruebas y los procedimientos utilizados para diagnosticar el colangiocarcinoma hiliar incluyen:
- Pruebas por imágenes, como la tomografía computarizada y las imágenes por resonancia magnética (IRM)
- Análisis de sangre para verificar si hay niveles muy altos del marcador tumoral CA 19-9
Biopsia mediante colangiopancreatografía retrógrada endoscópica o ecografía endoscópica y análisis de laboratorio especializados con hibridación fluorescente in situ
Por lo general, los médicos no recomiendan recolectar células mediante una aguja que se introduce en la piel hasta el conducto biliar (biopsia transperitoneal). Esta técnica aumenta el riesgo de recurrencia del cáncer y puede hacer que no seas elegible para un trasplante de hígado.
Tratamiento
El mejor tratamiento para ti dependerá de la ubicación y del tamaño del colangiocarcinoma hiliar. El tratamiento por lo general incluye cirugía, trasplante de hígado o tratamientos complementarios para reducir el dolor y otros síntomas.
Cirugía
La cirugía de colangiocarcinoma hiliar involucra la extracción de lo siguiente:
- Cáncer
- Conductos biliares circundantes
- Partes del hígado
- Ganglios linfáticos cercanos
El cirujano luego conecta los conductos biliares restantes al intestino delgado para que la bilis pueda llegar al tracto digestivo y para ayudar a digerir los alimentos.
Trasplante de hígado
Algunas personas con colangiocarcinoma hiliar en etapa temprana pueden considerar un trasplante de hígado. Por lo general, se utilizan quimioterapia y radiación antes del trasplante de hígado para eliminar la mayor cantidad posible de células cancerosas antes de la cirugía.
Este tratamiento comúnmente comprende lo siguiente:
- Quimioterapia intravenosa con un medicamento que hace que las células sean más vulnerables a la radiación
- Radioterapia de haz externo
- Radiación interna (braquiterapia) mediante pequeños cables que se colocan cerca del cáncer y liberan radiación lentamente
- Quimioterapia en forma de píldoras hasta la cirugía para el trasplante
- Cirugía de invasión mínima para buscar signos de que el cáncer se ha propagado
- Trasplante de hígado de un donante si hay uno disponible
Es posible que debas someterte a pruebas de rutina para evaluar tu salud mientras esperas un trasplante de hígado para asegurar que estés en buenas condiciones de salud para la cirugía.
Quimioterapia
Las personas con colangiocarcinoma hiliar avanzado que no pueden someterse a una cirugía o a un trasplante de hígado pueden recibir quimioterapia para retrasar el crecimiento del cáncer.
Estrategias de afrontamiento y apoyo
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?