All About Pancreatic Cancer

What is the pancreas?

The pancreas is a pear-shaped gland. It is about six inches long, and found in the abdomen, between the stomach and the spine. It has three parts. The widest part is called the head, the middle section is the body, and the thin end is called the tail. The pancreas makes hormones such as insulin. Insulin helps control blood sugar levels and enzymes. These enzymes help digest food. These enzymes move through ducts of the pancreas and empty into the common bile duct. The common bile duct carries the enzymes into the bowel.

What is pancreatic cancer?

Cancer is when cells grow in an uncontrolled way. This can lead to a tumor being made. If these tumors spread to lymph nodes or other organs, they are called metastatic. About 70% of pancreatic cancers happen in the head of the pancreas. Most pancreatic cancers start in a duct and the ducts carry enzymes. They are called pancreatic adenocarcinomas. There are other types of pancreatic cancer that don’t start in the ducts. These make up a small number of pancreatic cancers.

What causes pancreatic cancer and am I at risk?

About 60,000 new cases of pancreatic cancer are diagnosed in the United States each year. Pancreatic cancers represent 3% of all cancer cases in the United States. Pancreatic cancers happen more in people between the ages of 66-84. It is rarely seen in those under 40 years old. It is slightly more common in men than women. 

Cigarette smoking can increase your risk of pancreatic cancer. Heavy alcohol users may also be at a higher risk of getting pancreatic cancer. It is more common in people with diabetes and obese people, but this link is not yet well understood. Certain workplace exposures may also increase risk. These include chemists, coal, gas, and metal workers, and those employed in industries where pesticides are used more often. 

Your risk may be higher if your mother, father, or siblings have had pancreatic cancer. A family history of breast or colon cancer also increases risk. This is due to inherited mutations in cancer-causing genes (changes that allow cancer to develop), including the BRCA2 mutation.

How can I prevent pancreatic cancer?

No one really knows what causes the disease so it is hard to prevent it. Quitting smoking decreases your risk. Maintaining a healthy body weight can also help prevent pancreatic cancer.  Be sure to inform your healthcare providers of any family history of pancreatic cancer.

What screening tests are used for pancreatic cancer?

There are no standard screening tests for pancreatic cancer.

If you have known familial genetic syndromes that are linked to pancreatic cancer including hereditary pancreatitis, Peutz-Jeghers syndrome (PJS), BRCA2 mutations, and familial atypical multiple mole melanoma (FAMMM) syndrome, you should talk about screening with your healthcare provider. This screening would begin 10 years earlier than the time when the disease is typically seen. For example, people with PJS tend to get pancreatic cancer at an average age of 40, so screening would begin at age 30. Those known to have these genetic syndromes should be followed by specialists that treat the syndrome. Tests that are used for screening include CT scan, MRI, and EUS (endoscopic ultrasound).

Researchers have discovered genetic changes present in pancreatic cancer. These genes are found in stool, bowel and enzyme fluid, bile, and blood. Researchers are looking at these genes as a potential way to screen people for pancreatic cancer in the future.

What are the signs of pancreatic cancer?

The signs of early-stage pancreatic cancer are not specific to the disease. They can be thought to be other health issues by both patients and providers. More specific symptoms tend to start after the tumor has grown and spread to other organs or block the bile ducts. Symptoms include 

• Weight loss.
• Loss of appetite.
• Jaundice (a condition that causes yellowing of the eyes and skin and darkening of urine).
• Pain in the upper abdomen or back.
• Weakness.
• Blood clots.
• Nausea and vomiting. 

These symptoms can vary depending on where the tumor is found in the pancreas (head, body, or tail). Newly developed diabetes can be a sign in some people. Diabetes is caused by the pancreas not making insulin. 

How is pancreatic cancer diagnosed?

If your provider thinks you may have pancreatic cancer there are many tests that can be done to make a diagnosis. These include:

• CT Scan (often a multiphase or pancreatic protocol CT): A CT can find a tumor in the pancreas, enlarged lymph nodes (which may indicate tumor involvement), tumors in the liver, or obstructions (blockage) of the bile duct.
• Ultrasound: The use of a device that emits sound waves, which bounce off the organs, producing echoes that are used to create an image of the organ. This can be done on the outside of the abdomen (called transabdominal ultrasound) or from inside the bowel (called endoscopic ultrasound or EUS). 
• MRI.
• PET/CT scans.

If you have jaundice, your healthcare provider may want to do a test to find out where the bile duct is blocked and if this blockage is caused by a tumor or another issue. These tests include endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC). 

In ERCP, a tube is passed through the mouth down the throat to the bowel, where a small catheter is placed into the bile and pancreatic ducts. Dye is injected and x-rays are taken. The x-rays will show where the blockage is and what it is caused by. In PTC, dye is injected through a needle that is placed through the skin, into the liver. The dye moves into the bile ducts, again allowing the blockage and its cause to be seen with an x-ray. In some cases, a small sample of tissue (biopsy) may be removed during these procedures to be looked at by a pathologist.

Some with pancreatic cancer may have a higher level of carbohydrate antigen 19-9 (CA 19-9), but this is not found in all people and may be caused by other things. A CA 19-9 can be helpful to confirm the diagnosis as well as can be watched during treatment to measure how well the treatments are working. Another tumor marker related to pancreatic cancer that may be looked at includes carcinoembryonic antigen (CEA).

How is pancreatic cancer staged?

After these tests are done, the stage of the cancer can be determined. The staging of cancer describes how much cancer has grown within the pancreas as well as if it has spread. This is very important in deciding treatment. The staging system used to describe pancreatic tumors is the "TNM system", as described by the American Joint Committee on Cancer. The TNM systems are used to describe many types of cancers. They have three components

• T-describes the size/location/extent of the "primary" tumor in the esophagus.
• N-describes if the cancer has spread to the lymph nodes
• M-describes if the cancer has spread to other organs (i.e.-metastases). 

Pancreatic tumors are also called resectable or unresectable, meaning whether or not they can be removed with surgery. This depends on not only the size and spread but whether or not other body parts (for example, major blood vessels or organs) are involved.

The staging system is very complex. The entire staging system is outlined at the end of this article. Though complicated, the staging system helps healthcare providers determine the extent of the cancer, and in turn, make treatment decisions for your cancer. 

How is pancreatic cancer treated?

Tumors that are localized (haven’t spread beyond the pancreas) may be removed during surgery (resected). This surgery, called a Whipple procedure or pancreaticoduodenectomy, is a major, and recovery can be hard. This surgery is only used if you have a small, resectable tumor. Studies have found that patients do better when they have surgery at a regional referral center that does more than 40 Whipple procedures per year. You will need chemotherapy and radiation therapy after surgery (called adjuvant therapy) to try to prevent the tumor from coming back (recurrence).

Some tumors that have not spread to other organs can’t be removed with surgery. This is called locally advanced pancreatic cancer. In these cases, chemotherapy and radiation are used. Radiation is used to lessen the risk of the tumor coming back and chemotherapy is used to treat any cancer cells in the rest of the body. Used together they lessen the risk of metastases. Chemotherapy medications are used to both kill cancer cells and make cells more sensitive to radiation (called radiosensitization). In some cases, the chemotherapy and radiation make the tumor small enough that you may be able to have surgery later.

In the majority of patients with locally advanced cancer, treatment consists of chemotherapy (either fluorouracil (5-FU) or gemcitabine with radiation therapy). Other chemotherapy agents that may be used include cisplatin, capecitabine, albumin-bound paclitaxel, irinotecan, docetaxel and oxaliplatin. These agents are often used together to create a regimen. 

Targeted therapies work by targeting a specific pathway that helps a tumor grow. Erlotinib may be used with gemcitabine. Pembrolizumab is used as a treatment in unresectable or metastatic pancreatic cancers with an MSI-H (metastatic microsatellite instability-high) or dMMR (mismatch repair-deficient) tumor marker. Larotrectinib and entrectinib are used in tumors with an NTRK gene fusion. Olaparib is used in people with BRCA1/2 mutations. 

When the cancer has spread to other organs (metastasized), people will get chemotherapy alone or palliative care measures. Palliative care aims to improve quality of life by controlling pain and other symptoms. Palliative care can consist of pain medications, radiation therapy, or nerve blocks to control pain, and biliary stents to relieve symptoms of a bile duct obstruction.  Palliative care also provides psychological and spiritual support.

Clinical Trials

Clinical trials are extremely important in furthering our knowledge of this disease. It is through clinical trials that we know what we do today, and many exciting new therapies are currently being tested. Talk to your healthcare provider about participating in clinical trials in your area. You can also explore currently open clinical trials using the OncoLink Clinical Trials Matching Service.

Follow-up Care and Survivorship

After finishing treatment you will be followed closely with CT scans and tumor marker levels (CA 19-9) for any sign of recurrence. You should follow up with your healthcare provider every 3 months for the first two years after therapy is done and every 6-12 months after the first two years.

Fear of recurrence, the financial impact of cancer treatment, employment issues, and coping strategies are common emotional and practical issues experienced by pancreatic cancer survivors. Your healthcare team can identify resources for support and management of these practical and emotional challenges faced during and after cancer.

Cancer survivorship is a relatively new focus of oncology care. With nearly 17 million cancer survivors in the U.S. alone, there is a need to help patients transition from active treatment to survivorship. What happens next, how do you get back to normal, what should you know and do to live healthy going forward? A survivorship care plan can be a first step in educating yourself about navigating life after cancer and helping you communicate knowledgeably with your healthcare providers. Create a survivorship care plan today on OncoLink.

Resources for More Information

Pancreatica-Confronting Pancreatic Cancer

Provides pancreatic cancer educational information, and a “buddy” program to match individuals with trained pancreatic cancer survivors.

www.pancreatica.org

Pancreatic Cancer Action Network

Offers free educational information, 1-to-1 patient support, a telephone hotline, clinical trials information, advocacy and fundraising for pancreatic cancer research and patient services.

www.pancan.org

Appendix: Complete Pancreatic Cancer Staging

AJCC, Cancer Staging Manual, 8^th Edition