Abramson Cancer Center

Risk factors can increase the chance of getting cancer. There are different kinds of risk factors. Some risk factors for cancer, like age and family history, cannot be prevented.

Patients who feel they are at risk may benefit from consulting with a risk assessment specialist within one of Penn Medicine’s programs at the Abramson Cancer Center or the Joan Karnell Cancer Center at Pennsylvania Hospital.

Bile Duct Cancer Risk Factors

Risk factors increase the chance of developing bile duct cancer. Having a risk factor, or even several, does not always mean that someone will get cancer.

• Inflammatory diseases of the bile ducts. Studies show that people with inflammatory diseases of the bile ducts such as ulcerative colitis and primary sclerosing cholangitis (PSC) may be at increased risk for developing cholangiocarcinomas.
• Chronic liver disease. People with chronic liver disease such as hepatitis B or C, alcoholic liver disease or cirrhosis from other causes may be at an increased risk.
• Parasitic liver disease. Parasitic liver disease, such as an infection with the Chinese liver fluke, may increase the risk for developing cholangiocarcinomas.
• Congenital liver abnormalities. Choledochal cysts such as Caroli’s diseases have been associated with an increased risk for cholangiocarcinomas.
• Chemical exposure. Exposure to the chemical thorotrast, a form of thorium dioxide, has been linked to the development of cholangiocarcinoma. Thorotrast was banned in the United States in the 1950s, but its effects may show up as late as 30 to 40 years after exposure.

Bile Duct Cancer Prevention

Most risk factors for bile duct cancer cannot be prevented, such as pre-existing conditions. However, there are some things people at risk can do to prevent cholangiocarcinomas:

• Control weight. Maintaining a healthy weight through diet and exercise can help prevent cancer. 

Bile Duct Cancer Risk Assessment

Penn Medicine offers programs for patients who want to determine their risk of developing gastrointestinal cancer through Abramson Cancer Center.

These programs offer knowledge about the presence of genetic risk factors for cancer and provide patients with important, sometimes life-saving options.

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Most symptoms from cholangiocarcinoma arise from an obstruction within the bile ducts. Symptoms of bile duct cancer include:

• Jaundice, or yellowing of the skin and whites of the eyes
• Abdominal pain, particularly in the right upper portion of the abdomen
• Itching skin
• Weight loss without dieting
• Fever
• Gray or clay colored stool
• Dark urine

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Staging systems provide doctors with a common language for describing tumors. After cancer is first diagnosed, a series of tests are used to investigate the extent of the cancer and to see whether it has spread to other parts of the body from where it started. Staging is a way of recording the size and growth of a cancer, and determining the plan for treatment. By understanding the stage of their cancer, patients can make informed decisions about their treatment.

Staging bile duct cancer attempts to discover the following:

• The size of the tumor.
• Whether the cancer has spread to nearby tissues.
• Whether the cancer has spread to other parts of the body.

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After being diagnosed with bile duct cancer, patients at the Abramson Cancer Center may be evaluated through the Gastrointestinal Cancer Evaluation Center (GICEC). The center provides patients with expert support and evaluation to discuss treatment options and individualized treatment plans.

Patients with bile duct cancer are treated by a multidisciplinary team of cancer specialists who see more patients with gastrointestinal (GI) cancers in one year than many doctors see in their careers.

The physicians of Penn Medicine’s GI cancer program are nationally recognized for their expertise in the diagnosis and treatment of cancer.

Penn’s GI cancer treatment team includes:

• Nationally recognized medical experts with years of experience in diagnosing and treating GI cancer.
• Nurses with advanced training and experience in caring for patients with GI cancer.
• Oncology Navigation Specialists to help patients navigate the health system.
• Registered dietitians to provide nutrition counseling.
• Cancer counselors to provide individual or family counseling for issues many cancer patients face.
• Rehabilitation therapists who specialize in the diagnosis and treatment of physical effects from cancer and its treatment.

Patients at Penn Medicine can benefit from participating in clinical trials that take place at Penn. Patients interested in learning more about clinical trials should speak with someone on their treatment team.

Oncology Navigation Specialists

Every step of cancer treatment – from cancer diagnosis, to surgery and treatment to forming a survivorship plan – comes with different needs and issues that should be addressed.

Oncology Navigation Specialists at the Abramson Cancer Center are available to make a patient’s experience as seamless as possible. As experts in navigating complex health care situations, Patient Navigation specialists serve as a consistent point of contact and a reliable source for advice, support and direction for patients and families. They can help in a variety of ways including; providing emotional support, identifying resources, and ensuring access to information, support services, educational programs and community resources.

Learn more about Patient Navigation specialists at the Abramson Cancer Center.

Penn Pancreatic and Biliary Center

Patients at Penn Medicine with bile duct cancer also benefit from the Penn Pancreatic and Biliary Center, one of the busiest pancreatic surgery programs in the United States.

Studies have shown successful treatment of pancreatic cancer requires accurate diagnosis and cancer surgeons who are experienced in complex pancreatic surgeries. The center combines that expertise with leading-edge research and technology, giving patients at Penn Medicine a better chance for positive outcomes.

Learn more about the Penn Pancreatic and Biliary Center.

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Currently, there are no standard bile duct cancer screening recommendations for the general public like mammograms for breast or colonoscopies for colon cancer. However, patients at increased risk may benefit from specific diagnostic testing, and this should be discussed with a gastroenterologist.

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When physicians suspect cancer, they may order several tests to make a diagnosis and stage the disease.

Bile duct cancer is most often diagnosed when patients show symptoms of a blockage or narrowing of the bile ducts.

• Medical history and physical exam. Physicians examine patients for jaundice, or yellowing of the skin, which may be a sign bile is not draining properly from the bile ducts.
• Lab tests. Lab tests can help a physician diagnose cancer, determine current organ function, learn how other organs may be affected and look for signs of reoccurrence. Lab tests can also look for elevated tumor markers such as carcinoembryonic antigen (CEA), cancer antigen (CA) 19-9 and liver function.
• Imaging tests. Pictures of the inside of the body can help find out whether a suspicious area might be cancerous, to learn how far cancer may have spread, and to help determine if treatment is working.
  • Endoscopic retrograde cholangiopancreatography (ERCP). ERCP is the diagnostic test most performed to diagnose bile duct cancer. Performed while the patient is under sedation, the procedure combines endoscopy and fluoroscopy to diagnose cancer. If cancer is suspected, the physician may biopsy the area or collect suspicious cells for further review.  
  • Endoscopic ultrasound (EUS).  Also performed under sedation, this procedure combines endoscopy and ultrasound to provide images of the bile ducts and surrounding structures, and may also be used for tissue acquisition.
  • Computed tomography (CT). Three-dimensional X-rays of the inside of the body are combined by a computer into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (dye) is used to provide better detail.
  • Magnetic resonance imaging (MRI). Magnetic fields are used to create detailed images of the body. A contrast medium may be injected into a vein to create a clearer picture.
  • Percutaneous transhepatic cholangiography (PTC). A thin needle is inserted via the liver into the biliary tree and dye is injected before an X-ray is taken. A stent may be left to drain bile if a blockage is found.
• Biopsy. If other tests find a change that is possibly cancer, a sample of the suspicious area is removed and viewed under a microscope. A biopsy is the only way to tell if cancer is really present. 
  • Brush cytology. Brush cytology is a type of procedure that collects suspicious cancer cells. During an ERCP, a physician may use a small brush passed through the endoscope to collect cells for the lab.
  • Cholangioscopy. During an ERCP, a smaller endoscope is threaded through the larger one. The scope is inserted into the bile duct and, using a tiny camera, the physician can take a directed biopsy of the suspicious area. This procedure removes a larger piece of tissue, and may provide a more accurate diagnosis.
• Laparoscopy. A minimally invasive surgical procedure allows the physician to view the lymph nodes and other organs to see if cancer has spread. Biopsies may be taken of the organ tissue.

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Staging systems provide doctors with a common language for describing tumors. After cancer is first diagnosed, a series of tests are used to investigate the extent of the cancer and to see whether it has spread to other parts of the body from where it started. Staging is a way of recording the size and growth of a cancer, and determining the plan for treatment. By understanding the stage of their cancer, patients can make informed decisions about their treatment.

• Stage 0. Cholangiocarcinomas only involve the lining of the bile duct and have not spread to lymph nodes or other organs. Also called carcinoma in situ.
• Stage I. One of the following:
  • IA. Cancer is found in the bile duct only.
  • IB. Cancer has spread through the wall of the bile duct
• Stage II. One of the following:
  • IIA. Cancer has spread to the liver, gallbladder, pancreas and/or the right or left branch of the hepatic artery or the right or left branch of the portal vein.
  • IIB- Cancer has spread to nearby lymph nodes and is found in the bile duct, has spread through the wall of the bile duct, or has spread to the liver, gallbladder, pancreas, and/or right or left branches of the hepatic artery or portal vein.
• Stage III. Cancer that has spread and one of the following:
  • To the main portal vein or to both the right and left branches of the portal vein.
  • To the hepatic artery.
  • To other nearby organs and tissues.
• Stage IV. Tumor that has spread to other distant organs.

A physician may also classify the disease as one of the following:

• Local. The bile duct cancer lies within the bile duct and can be completely removed surgically. There is no evidence of any spread to areas outside of the bile duct.
• Locally advanced. The cancer is still confined to the area around the bile duct, but does affect nearby organs, arteries, and/or veins. There is no evidence of spread to any distant areas of the body.
• Metastatic. The tumor has spread beyond the area of the bile duct to reach distant areas of the body. It is unlikely that surgery can remove the cancer.

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A subset of patients with bile duct cancer may be candidates for curative surgery. Unfortunately, most cases of bile duct cancer are not operable.

Bile Duct Resection

In a bile duct resection, some or all of the bile duct may be removed if the tumor is small. A new duct is made by connecting the duct openings in the liver to the intestine. Lymph nodes may be removed to check for cancer.

Partial Hepatectomy

A partial hepatectomy removes the part of the liver near the bile duct where cancer is present. The part may be a wedge, an entire lobe, or a larger part of the liver along with some surrounding tissue.

Whipple procedure

A Whipple procedure, or pancreaticoduodenectomy, is a complex procedure in which the gallbladder, common bile duct, part of the duodenum, and the head of the pancreas are removed. Enough of the pancreas is left to continue to produce digestive enzymes. Studies have confirmed that the success of the procedure is linked to the experience of the surgeon performing the procedure.

Surgical Biliary Bypass

If a tumor is blocking the small intestine and bile is building up in the liver, a biliary bypass may be performed. The gallbladder or bile duct is cut and sewn directly to the small intestine to create a new pathway around the blockage. This procedure is not designed to cure bile duct cancer, only relieve symptoms.

Endoscopic Stent Placement

If the tumor is blocking the bile duct, a stent may be placed employing ERCP or interventional radiology to drain bile that builds up in the area. This procedure is not designed to cure bile duct cancer, only relieve symptoms.

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Radiation oncologists at Penn Medicine are recognized for techniques that target radiation precisely to the disease site while sparing nearby tissue.

Proton therapy at Penn Medicine is now being used to treat upper gastrointestinal cancers and recurrent tumors in the digestive tract. Penn Medicine is one of the only facilities in the country treating gastrointestinal cancers in this way.

Radiation therapy uses high-energy radiation to kill cancer cells. A radiation therapy schedule usually consists of a specific number of treatments given over an extended period of time.

3-D Conformal Radiation Therapy

The imaging technology used by radiation oncologists at Penn Medicine helps shape the radiation treatment beam to the shape of the tumor. Conformal radiation therapy gives doctors more control when treating tumors.

Special computers use CT imaging scans to create 3-D maps of the location of the cancer in the body. The system permits the delivery of radiation from several directions, and the beams can then be shaped, or conformed, to match the shape of the cancer. Conformal radiation therapy limits radiation exposure to nearby healthy tissue as well as the tissue in the beam's path.

Intensity-Modulated Radiation Therapy (IMRT)

IMRT is an advanced type of radiation therapy that uses a computer-controlled device, called a linear accelerator, to deliver precise doses of radiation to tumors or specific areas within the tumors. Radiation therapy, including IMRT, may stop cancer cells from dividing and growing, thus slowing or stopping tumor growth.

Using 4-D computed tomography (CT) images along with computerized dose calculations, IMRT allows for the radiation dose to conform more precisely to the shape of the tumor by controlling, or modulating, the intensity of the radiation beam while tracking any movement of the tumor. The therapy allows higher radiation doses to be delivered to areas within the tumor while minimizing the dose to the surrounding area.

Volumetric-Modulated Arc Therapy (VMAT)

VMAT is a type of specialized intensity modulated radiation therapy, or IMRT. VMAT delivers radiation by rotating the radiation machine, through one or more arcs while radiation is continuously delivered.

VMAT allows Penn radiation oncologists to treat complex cancers while minimizing exposure to surrounding healthy tissue. VMAT shortens radiation delivery time, and offers patient more comfort because it does not require patients to lie completely still for long periods of time.

Penn radiation oncologists use RapidArc™ radiotherapy technology to treat hard to reach tumors.

Proton Therapy

Penn Medicine is the only health care facility in the country that is using proton therapy to treat recurrent gastrointestinal tumors and tumors in the upper digestive tract in clinical trials.

Penn Medicine's Roberts Proton Therapy Center is the largest and most advanced facility in the world for this precise form of cancer radiation. Patients have access to one of the most sophisticated weapons against cancer, seamlessly integrated with the full range of oncology services available at Penn Medicine. Proton therapy is external beam radiotherapy in which protons are directed at a tumor.

The radiation dose that is given through protons is very precise, and limits the exposure of normal tissues. This allows the radiation dose delivered to the tumor to be increased beyond conventional radiation. The result is a better chance for curing cancer with fewer harmful side effects.

Proton therapy, like all forms of radiation therapy, works by aiming the energized particles, in this case protons, onto the target tumor. The particles damage the DNA of cells, ultimately causing their death. Unlike X-rays, protons can be manipulated to release most of their energy only when they reach their target. With more energy reaching the cancerous cells, more damage is administered by each burst of radiation.

Brachytherapy

Radiation oncologists at Penn Medicine use both internal and external forms of radiation therapy to treat cancer. Brachytherapy is an internal therapy in which the radiation source is placed inside the body.

Brachytherapy for bile duct cancer involves placing radioactive wires inside tubes in small sealed holders in the body. The implants are left in the body for only a short time. This allows the doctor to give a high dose of radiation to a smaller area than is possible with external radiation treatment.

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Penn Medicine specializes in a team approach to treatment with multidisciplinary care and innovative approaches that use chemotherapy to target tumors prior to and after surgery.

Chemotherapy can also be delivered to potentially enhance the effectiveness of radiation therapy.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells. It is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is usually delivered intravenously through an IV, or orally by pill.

Biologic therapy

Biologic therapy, also called targeted therapy, uses the patients' own immune system to target cancer cells. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer, and are delivered orally by pill or intravenously. This type of cancer treatment is also called immunotherapy.

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Today, more and more people are surviving cancer. Clinical trials benefit patients with access to breakthrough therapies and treatments. These new advances in cancer treatment are occurring every day at Penn Medicine, giving patients hope that even greater discoveries lie ahead. Through clinical trials:

• Diagnosing cancer has become more precise.
• Radiation and surgical techniques have advanced.
• Medications are more successful.
• Combinations of medical, surgical and radiation therapy are improving treatment effectiveness and enhancing outcomes.
• Strategies to address the late effects of cancer and its treatment are improving quality of life.

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In addition to standard treatments and clinical trials, patients at Penn Medicine may wish to add additional therapies and treatments such as massage therapy, acupuncture and art therapy. These therapies do not have curative intent, and are designed to complement, not replace, standard treatments.

Integrative Medicine and Wellness Programs

At Penn Medicine, integrative medicine and wellness services can supplement traditional cancer treatments such as chemotherapy, surgery and radiation therapy. While conventional medicine plays a critical role in eradicating cancer, integrative medicine and wellness programs offer patients and their families ways to enhance the quality of their lives, minimize or reduce the side effects of cancer and cancer treatment, and promote healing and recovery.

Cancer specialists at Penn Medicine are knowledgeable and supportive of complementary cancer treatments. The cancer team works with patients and families to integrate these supportive programs into the overall care plan, while ensuring the safety and health of patients.

The Abramson Cancer Center’s range of integrative supportive services is designed to help patients cope with the cancer experience and improve their overall sense of well-being. Services include:

• Acupuncture
• Diet and nutrition
• Mindfulness-based stress reduction therapy
• Reiki therapy
• Yoga
• Exercise and rehabilitation

The Joan Karnell Cancer Center at Pennsylvania Hospital offers a variety of supportive care programs for patients and families, from diagnosis through survivorship. These programs are available at no cost to the patients treated at Pennsylvania Hospital, and some are open to patients treated elsewhere. These services include social work counseling, nutrition counseling, psychological counseling and spiritual counseling.

The Cancer Appetite and Rehabilitation Clinic focuses on patients with loss of appetite and weight.

The Supportive Care Clinic helps to manage cancer related symptoms. Integrative support programs include:

• Music therapy
• Art therapy
• Shiatsu massage
• Mindfulness meditation

Support groups and educational programs are available at Pennsylvania Hospital throughout the year.

Palliative Care

Palliative care provides medical and non-medical interventions to ease the symptoms of cancer and its treatment. Palliative care includes physical, emotional and spiritual care that can enhance the quality of life for cancer patients.

Palliative care can be used to complement traditional cancer therapies, or can be used to improve quality of life when curative therapies are no longer an option.

Palliative care is an approach to patient care that can be integrated with curative therapies at any point from diagnosis to survivorship or end-of-life care.

Palliative care services include palliative chemotherapy, radiation therapy and surgery as well as psychological counseling, art therapy and support groups for patients and families. The goals of palliative care are to enhance the quality of life for cancer patients and their families and provide emotional and spiritual support to enhance personal growth.

Palliative care services are offered at Pennsylvania Hospital, and at the Hospital of the University of Pennsylvania.  

Penn Home Care and Hospice Services

Penn Home Care and Hospice Services offer a full range of home health care needs by partnering three top-level home health care services under one roof:

• Penn Wissahickon Hospice
• Penn Care at Home
• Penn Home Infusion Therapy

Penn Home Care and Hospice Services offer an array of specialized therapies and medications for patients with cancer and cancer-related conditions.

Oncology Navigation Specialists

Every step of cancer treatment – from cancer diagnosis, to surgery and treatment to forming a survivorship plan – comes with different needs and issues that should be addressed.

Oncology Navigation Specialists at the Abramson Cancer Center are available to make a patient’s experience as seamless as possible. As experts in navigating complex health care situations, patient support specialists serve as a consistent point of contact and a reliable source for advice, support and direction for patients and families. They can help in a variety of ways including; provide emotional support, identify resources, and ensure access to information, support services, educational programs and community resources.

Learn more about Patient Navigation specialists at the Abramson Cancer Center.

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At the end of treatment, bile duct cancer patients should schedule a visit with their oncologist and nurse practitioner. At this time, patients are provided with a summary of the treatment received as well as a plan for follow-up care.

Long-term cancer survivors should speak to their oncologists and nurse practitioner about scheduling a one-time survivorship-focused consultation with a nurse practitioner or physician. Annual or more frequent follow-up appointments are also available.

Penn cancer providers work one-on-one with patients to develop survivorship care plans. Since every cancer is unique, the plans are tailored to the patients. 

The survivorship care plan includes information on:

• Potential long-term or late side effects of cancer treatment, the symptoms and treatment.
• Recommendations for cancer screening for disease recurrence or a new cancer.
• Psychosocial effects, including relationships and sexuality.
• Planning follow-up visits.

A survivorship care plan encourages patients to review the information with their health care team and become active participants in their follow-up care.

Penn also offers cholangiocarcinoma patients support programs and groups to enhance their care.

Oncology Navigation Specialists

Every step of cancer treatment; from a cancer diagnosis, to surgery and treatment to forming a survivorship plan; comes with different needs and issues that should be addressed.

Oncology Navigation Specialists at the Abramson Cancer Center are available to make a patient’s experience as seamless as possible. As experts in navigating complex health care situations, patient support specialists serve as a consistent point of contact and a reliable source for advice, support and direction for patients and families. They can help in a variety of ways including; provide emotional support, identify resources, and ensure access to information, support services, educational programs and community resources.

Learn more about patient navigation specialists at the Abramson Cancer Center.

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Penn Medicine's Living Well After Cancer™ Program is a nationally recognized program that focuses on issues facing cancer survivors. In 2007, the Abramson Cancer Center was designated a LIVESTRONG ™ Survivorship Center of Excellence Network.  The Abramson Cancer Center is only one of eight LIVESTRONG centers in the United States, and is the only LIVESTRONG Survivorship Center of Excellence in the Philadelphia region. The program focuses on survivorship, a distinct phase of care.

Prescription for Living: The Cancer Survivorship Program at the Joan Karnell Cancer Center at Pennsylvania Hospital, provides patients with a summary of important information about specific cancer diagnoses and treatments, as well as follow-up information and steps to take towards recovery, supportive care and education to help patients adjust to their lives as cancer survivors.

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Penn offers cancer patients support programs and groups to enhance their survivorship care plans.

The Abramson Cancer Center produces a wide range of materials and hosts activities that provide education and support to address key areas of concern including survivorship for cancer patients and their family and friends.

Focus On: Gastrointestinal Cancers is a day-long conference that addresses issues for patients with gastrointestinal cancer, as well as their loved ones.

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