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Be empowered by knowledge. Learn everything you need to know about a pancreatic cancer diagnosis.

Pancreatic Cancer Overview

The Pancreas

The pancreas is an important organ of the digestive system. It is often referred to as the “hidden organ” because it is located deep within the abdomen, behind the stomach and in front of the spine.

About six to eight inches long in an adult, the organ contains thin tubes that come together like veins in a leaf. These tubes join to form a single opening into the intestine that is located just beyond the stomach.

The pancreas produces juices and enzymes that flow through these tubes into the intestine, where they mix with food. The enzymes digest fat, protein, and carbohydrates so they can be absorbed by the intestine. Pancreatic juices, therefore, play an important role in maintaining good health.

The pancreas also produces insulin, which is picked up by the blood flowing through the organ. Insulin is important in regulating the amount of sugar or glucose in blood.


A number of problems can occur in the pancreas. These include: 


Diabetes Mellitus 

Many cases of diabetes are caused by a deficiency of insulin. Insulin is needed to help glucose, which is a major source of energy, that enters the body’s cells. It is not known why insulin-producing cells in the pancreas die off. When they cease to function, glucose accumulates in the blood and eventually spills into the urine. These patients require daily insulin injections. More importantly, high blood glucose levels, over time, result in significant changes in blood vessels in the eyes, kidneys, heart, legs, and nerves. Damage to these vital organs represents the major risk for patients with diabetes. 

Acute Pancreatitis 

This condition occurs when the pancreas becomes quickly and severely inflamed. The major causes are: 
• Heavy alcohol ingestion 
• Gallstones or gallbladder disease 
• Trauma 
• Drugs 
• High blood fats (triglycerides) 
• Heredity 
• Unknown factors 

Binge alcohol drinking is a common cause of acute pancreatitis. Gallbladder disease, especially where a gallstone becomes lodged in the main bile duct next to the pancreas, also causes this condition. Accidents, such as the upper abdomen hitting the steering wheel during a car accident, can also cause pancreatitis. Certain drugs, such as diuretics, can produce the disorder as can extremely high blood fat levels (triglycerides). Heredity can also play a role. 

Finally, there are the occasional cases that occur for unknown reasons. In pancreatitis, the digestive enzymes of the pancreas break out into the tissues of the organ rather than staying within the tubes (ducts). Severe damage to the pancreas then results. 

Pancreas Enzyme Insufficiency 

Digestive enzymes from the pancreas are necessary to break down protein, fat, and carbohydrates in foods that are ingested. When there is a deficiency of these enzymes, nutrients are not broken down, resulting in malnutrition and weight loss.

This condition is called malabsorption because the intestine is unable to absorb these vital nutrients. The two major symptoms are diarrhea (frequently with fat droplets in the stool) and weight loss.

This condition can result from any cause of pancreatitis, including trauma and infection. Pancreatic enzymes can be taken by mouth to replace those no longer made by the pancreas. 

Pancreatic Tumors 

The pancreas, like most organs of the body, can develop tumors. Some of these are benign (not cancerous) and cause no problems.

However, some benign tumors can secrete hormones which, when present in high levels, have a damaging effect. For example, insulin can be secreted in excessive amounts and result in dangerously low blood sugar levels (hypoglycemia).

Another hormone called gastrin can stimulate the stomach to secrete its strong hydrochloric acid causing recurrent stomach and peptic ulcers, with many complications. Fortunately, there is much that can be done about these tumors. 

Cancer of the pancreas is a serious malignancy which is difficult to treat. Pancreatic cancer occurs most often in middle or older aged people, with the first symptom often being dull pain in the upper abdomen that may radiate into the back. At times, skin jaundice (yellow skin color) occurs when the bile duct, which carries yellow bile from the liver and through the pancreas, is blocked.

Surgery is the only effective form of treatment for pancreatic cancer.

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Pancreatic Cancer

The word “cancer” is used to describe a group of diseases in which the cells are abnormal, grow out of control, and can spread. 

Pancreatic cancer occurs when abnormal cells grow out of control in the tissue of the pancreas and form a tumour. A tumour is an abnormal growth of tissue in any part of the body.

Currently, there is no effective screening test to identify pancreatic cancer. As a result, pancreatic cancer is rarely diagnosed in the early stage. Patients are their own best advocate. Individuals must listen to their bodies


Pancreatic Cancer Types

It is important to understand that not all pancreatic cancers are the same and they should NOT be treated the same. If pancreatic cancer is suspected, a biopsy is required to confirm the diagnosis.

More than 95 percent of pancreatic cancers are classified as exocrine tumors. These tumours start in the exocrine cells of the pancreas, which produces enzymes and aid in the digestion of food.  Exocrine tumours are often aggressive and all are malignant. Adenocarcinomas account for the majority of exocrine tumours. 

Less than 5 percent of pancreatic tumours are classified as neuroendocrine (endocrine)  tumors (pancreatic NETs or PNETs). They may be benign or malignant and they tend to grow more slowly than exocrine tumors.  (Source: World Pancreatic Cancer Coalition) 


Exocrine Tumours

Endocrine Tumours
(Pancreatic Neuroendocrine Tumours PNETs)

Adenocarcinoma

Gastrinoma (gastrin)

Acinar Cell Carcinoma

Glucaganoma (glucagon)

Intraductal Papillary-Mucinous Neoplasm (IPMN)

Insulinoma (insulin)

Mucinous Cystadenocarcinoma

Somatostatinoma (somatostatin)

VIPoma (vasoactive intestinal peptide)

Nonfunctional Islet Cell Tumor (no hormones)

Metastasized: What does it mean?

A major concern when diagnosing a pancreatic cancer is whether or not the cancer has already metastasized (spread) outside the pancreas. The location of the metastases will determine whether the patient has local disease (contained in the pancreas and around the area of the tumor) or metastatic disease (the tumor has already spread to other organs). 

The location of the metastases will also determine whether the cancer can be surgically removed. There are certain sites that pancreatic cancer may spread to which may make surgery not possible. They are:

Lymph nodes
Metastases to lymph nodes does not automatically eliminate surgery as a possibility. The location of the affected lymph nodes makes a big difference. For example, the lymph nodes between the duodenum and the pancreas are a very common site of metastases. They are routinely removed during surgery. However, the spread of cancer to lymph nodes closer to the liver, may mean the tumor cannot be surgically removed. Typically, pancreatic cancer first metastasizes to regional lymph nodes, then to the liver, and, less commonly, to the lungs. In some cases, pancreatic cancer may invade surrounding internal organs (duodenum, stomach and colon).

Liver
Metastases to the liver are a common finding, especially with tumors in the tail and the body of the pancreas. If there is evidence of a metastases to the liver, surgery will usually not be an option.

Coeliac (Celiac) plexus
These are the nerves for the area of the pancreas and surrounding organs. These are the nerves that cause back pain when a growing tumor presses on them.
What Pancreatic cancer looks like

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Diagnosis

Pancreatic cancer is a difficult cancer to diagnose and treat. It is difficult to detect and symptoms often do not appear until the cancer has grown for quite some time. This means that an early diagnosis is only possible for about 10-20% of the population. About 40-50% of patients are diagnosed after the cancer has metastasized. 

If an individual is experiencing pancreatic cancer symptoms a variety of assessments can be ordered by a physician, to assist in a timely and accurate diagnosis.

Bloodwork

Unfortunately, there are no blood tests that can make a diagnosis of pancreatic cancer. However, patients presenting with possible early symptoms of pancreatic cancer should first have the following bloodwork done: 

  • CBC
  • Bilirubin
  • LFTs
  • INR

Ultrasound
An abdominal ultrasound will rule out other causes for symptoms such as gallstones. This assessment is typically inexpensive and does not have lengthy wait times in most areas of Canada. 


An ultrasound is a diagnostic exam that uses high frequency sound waves to produce real time images of the body. This is done by placing a transducer or wand-shaped device against the area being examined. Unlike x-ray, ultrasound does not use radiation; therefore it is considered a safe alternative for imaging pregnant women.


CT scan
A CAT scan ordered for possible pancreatic cancer should be performed with contrast. It can be referred to as a CT abdomen, pancreatic protocol.


This test may take up to 2 hours and can be done on an outpatient basis. A CT scan is a painless procedure. The only discomfort is lying down flat for a few minutes. When contrast is used, the individual may feel warm all over the body during the injection. This is a short duration, painless feeling. Others may complain of a metallic taste in the mouth. The CT scan can cause a claustrophobic feeling because of the closed space enclosure. 

It is estimated that this type of CT scan can diagnose about 98% of all pancreatic cancers and distant metastases.

Serum Tumor Markers
Several serum tumour markers are associated with pancreatic cancer. To date, no single marker has been found to be optimal for screening or diagnosis. Realistically, only some non-specific markers can lead to early diagnosis if further investigated:

  • CA19-9
  • CEA
  • LFTs
  • Bilirubin
  • Glucose level change


Biopsy
‘Tissue is the Issue’ (Dr. Daniel Rayson) The only definitive way to diagnose cancer is to directly visualize cancer cells (tissue samples) under a microscope. A biopsy may be performed when pancreatic cancer is suspected. 

If the CT scan detects a mass in the pancreas which is suspicious for a pancreatic cancer, patients should be referred for a biopsy.  

Approximately 10% of pancreatic cancers are other types of cancer including neuroendocrine cancers. Prognosis is much better than cancers that originate in exocrine tissue. Other types of cancers include lymphomas and metastatic cancers to the pancreas including renal cell carcinoma, breast cancer and colorectal cancer. Only a biopsy can be used to distinguish between the types of cancer. Following detection of a mass in the pancreas all patients should be referred as soon as possible for a tumor biopsy.

Additional tests

Additional tests which may be requested based on symptoms and accessibility and availability are:

ERCP
An Endoscopic Retrograde Cholangiopancreatography (ERCP) is a procedure that is used with a dye, to view the bile and pancreatic ducts for any blockages. During an ERCP, patients receive an anaesthetic to numb the throat and medication to make the patient sleepy. A thin tube is passed down the throat, through the stomach, and into the small intestine. The ERCP doctors can collect small particles of any tumours there may be, to be analyzed under the microscope.

An ERCP is especially helpful for patients with jaundice because a stent can be inserted and left in place to keep ducts open, often relieving the jaundice and its associated symptoms. 


MRI

Magnetic Resonance Imaging (MRI) is an imaging method which many people are familiar with. This procedure is painless and does not require the patient to drink anything or inject anything into the veins. MRI is widely used for imaging soft tissues in the body including the brain, nerves and muscles.

PET scans
Positron Emission Tomography, are increasingly read alongside CT or MRI scans. The combination (“co-registration”) gives both anatomic and metabolic information (i.e., what the structure is, and what it is doing biochemically). Because the two scans can be performed in immediate sequence during the same session, with the patient not changing position between the two types of scans, the two sets of images are more precisely registered. Therefore, areas of abnormality on the PET imaging can be more perfectly correlated with anatomy on the CT image.

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Early Signs and Symptoms

Pancreatic cancer often doesn’t cause any signs or symptoms in the early stages. Symptoms can be vague, non existent, or may come and go over a period of time and the severity of symptoms differ from patient to patient. Additionally, because the pancreas lies deep within the abdomen, a doctor performing an examination on a patient would not be able to feel a pancreatic tumour. All of this makes diagnosing pancreatic cancer challenging. 

The World Pancreatic Cancer Coalition recommends increased awareness of pancreatic cancer and the following symptoms that people may experience: 

  • Abdominal and mid-back pain
  • Unexplained weight loss
  • Yellow skin or eyes
  • Changes in stool
  • New-onset diabetes
  • Digestive problems -  (indigestion, nausea, lack of appetite)
  •  Mood change   

Additional Signs and Symptoms can include:

  • Blood Clots (Deep Vein Thrombosis)
  • Fatigue

Lastly, the early signs and symptoms of pancreatic cancer are often mistaken or misdiagnosed for other diseases such as: pancreatitis (inflammation of the pancreas), gallstones, irritable bowel syndrome (IBS) or hepatitis (inflammation of the liver). 


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Risk Factors

The cause of a pancreatic cancer diagnosis is usually unknown, however research studies have identified the risk factors below which may increase the likelihood of developing pancreatic cancer.

The World Pancreatic Cancer Coalition recommends increased awareness of the following pancreatic cancer risks: 

Inherited Genetic Mutations
Some of these disorders are:

  • BRCA gene mutation
  • cystic fibrosis
  • familial adenomatous polyposis
  • familial atypical multiple mole melanoma
  • Lynch syndrome
  • hereditary pancreatitis
  • PALB2 mutation
  • Peutz-Jeghers syndrome 

Family History of Pancreatic Cancer
People with two or more first-degree relatives who have had pancreatic cancer, a first-degree relative who developed pancreatic cancer before the age of 50, or an inherited genetic syndrome associated with pancreatic cancer may have an increased risk of developing pancreatic cancer.

Family History of Other Cancers
Risk of pancreatic cancer increases if there is a history of familial ovarian, breast, or colon cancer, hereditary pancreatitis or familial melanoma. 

Diabetes

Pancreatitis
Chronic and hereditary

Smoking
Smokers are two times more likely to develop pancreatic cancer than non-smokers. 

Obesity
People who are overweight are 20 times more likely to develop pancreatic cancer compared to those who are not overweight.

Race (ethnicity)
Pancreatic cancer is more prevalent (higher incidences) in African Canadians and Ashkenazi Jews, when compared with individuals of Asian, Hispanic or Caucasian descent. (Source: World Pancreatic Cancer Coalition) 

Age
Pancreatic cancer is most prevalent in individuals in their 60’s, 70’s and 80’s but is not limited to this age group.

Diet
Pancreatic cancer may be associated with high intakes of red meat and processed meats, but more research is needed. Additionally, a diet high in fruits and vegetables may decrease the risk.

Additional Risks

Medical Factors
Pancreatic cancer is more common in patients who:

  • have a history of cirrhosis (a chronic liver disease)
  • have chronic pancreatitis and individuals with hereditary pancreatitis
  • have in people who have long-standing diabetes (more than 5 years)
  • have surgery to the upper digestive tract.

Environmental Factors
Environmental factors may play a role, such as long-term exposure to certain chemicals, such as gasoline and related compounds, as well as certain insecticides, however further research needs to be completed to make this connection. 

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Genetics

Background Information:

DNA (deoxyribonucleic acid) is the molecule that contains each person’s unique genetic code. Nearly every cell in a human’s body has the same DNA. Half of our DNA is inherited from one biological parent and the other half is inherited from the other biological parent. 

Genes are segments of DNA that carry instructions that determine a person’s traits. Genes can also contribute to a person’s lifetime risk of developing a cancer. Most cancers are thought to develop from damage to the DNA of cells in the organ where the cancer develops (for example, the pancreas). One type of DNA damage is a change in a gene, which is called a gene mutation.

In 5-10% of pancreatic cancers, the gene mutations that lead to cancer development are inherited (the medical term is germline). That is, the gene mutations are passed on from one generation to the next. These hereditary cancers often occur as cancer syndromes where inherited gene mutations in a specific gene can give rise to different types of cancers, such as pancreatic cancer and breast cancer. 


Red Flags of Hereditary Cancer:

The following is a list of red flags for a hereditary cancer. These red flags are for all types of cancer and not specific for pancreatic cancer. 

  • A patient that is diagnosed with cancer at a young age (typically less than 50 years old)
  • A patient with multiple blood relatives that have the same type of cancer or cancers that are part of a cancer syndrome 
  • A patient that has multiple cancer diagnoses (such as breast and pancreatic cancer)
  • Specific types of cancer (for example, male breast cancer) have a higher association with hereditary causes
  • Certain ethnic groups and populations have a higher chance of having a germline gene mutation that is associated with cancer risk. For example, germline mutations in the BRCA1 and BRCA2 genes are more common in people of Ashkenazi Jewish descent and in the French-Canadian population

Germline Gene Mutations Associated with Pancreatic Cancer:

Germline mutations in the following genes are associated with pancreatic cancer risk. There are additional genes that have been implicated in hereditary pancreatic cancer risk, but they are less well established. 


  • Homologous DNA Repair Genes (BRCA1, BRCA2, PALB2): These genes are associated with breast, ovarian, prostate and pancreatic cancers. Germline BRCA2 gene mutations are the most frequent germline gene mutations seen in patients with pancreatic cancer, occurring in 3-10% of patients.
  • ATM: Individuals with a germline ATM mutation are at increased risk of developing certain cancers such as breast, prostate and pancreatic cancers.
  • CDKN2A: Germline mutations in the CDKN2A gene are best known for their association with familial malignant melanoma, but they also increase the risk of lung, pancreatic and breast cancers.
  • Mismatch DNA Repair Genes (MLH1, MSH2, MSH6, PMS2): Germline mutations in these genes are mostly known for causing Lynch Syndrome, which is also called Hereditary Non-Polyposis Colon Cancer, but they are also associated with increased pancreatic cancer risk. 
  • STK11: Germline mutations in STK11 are associated with Peutz-Jeghers Syndrome, which is a syndrome characterized by specific types of polyps in the digestive tract, called hamartomatous polyps. However, this syndrome is also associated with an increased risk of several cancers, including cancers of the breast, colon, rectum, pancreas, stomach, testicles, ovaries, and lung.
  • TP53: Germline mutations in TP53 cause Li-Fraumeni syndrome, which is associated with a wide range of cancers, including pancreatic cancer.
  • PRSS1, SPINK1: Germline mutations in these genes are associated with hereditary pancreatitis and pancreatic cancer.

Familial Pancreatic Cancer:

Pancreatic cancer has been observed to run in the families of around 10% of patients with pancreatic cancer. Some of these families will have a germline mutation in one of the genes that are associated with hereditary pancreatic cancer.  Familial pancreatic cancer (FPC) is used to describe families where there are multiple relatives diagnosed with pancreatic cancer, but there is no germline mutation in a known pancreatic cancer gene. The causes of FPC are an important area of current research.


FPC is identified when there are:

  • 2 or more members of a family who are first-degree relatives (such as parents, children, or siblings) who have been diagnosed with pancreatic cancer

or

  • 3 or more close relatives from the same side of the family who have been diagnosed with pancreatic cancer

Genetic Counselling and Genetic Testing:
Genetic Counselling:

A genetic counsellor will contribute to a pancreatic cancer patient’s care by evaluating their family history and discussing the role of genetic testing. A genetic counsellor is a healthcare professional with advanced training in medical genetics and counselling, who interprets genetic test results and provide guidance and support to patients seeking more information about issues such as (NSGC.org):

  • How inherited diseases and conditions might affect them and their families
  • How family and medical histories impact disease occurrence or recurrence
  • Which genetic tests may or may not be right for them, and what those tests may or may not tell
  • How to make the most informed choices about healthcare conditions

A genetic counselling session for cancer genetics will generally include:

  • Risk assessment, based on:
  • Personal and family history
  • Medical records
  • Genetic education and discussion of genetic testing options
  • Discussion about the potential impact of genetic test results on the patient and their relatives

Depending on the genetic counsellor’s assessment, and provincial/territorial practices, you may or may not be offered genetic testing. A genetic counsellor will never obligate you to have genetic testing. If you are not eligible for testing, other options may include research participation.

Benefits of germline genetic testing:

If you are a pancreatic cancer patient, identifying a germline mutation may have an impact on your treatment options. For example, patients with certain germline mutations may respond better to specific chemotherapy regimens or may be eligible for certain clinical trials.

If you are found to have a germline mutation, this information may allow genetic counselling and testing of your relatives to see if they also carry the gene mutation. If your relatives are found to carry a germline gene mutation, they may be eligible for pancreatic cancer screening. In Canada, screening tests for pancreatic cancer are currently being performed mostly as part of research studies. Since these germline mutations can also increase their risk of developing other types of cancer, they may benefit from risk-reducing interventions for these related cancers. For some cancers, there are established screening tests (for example, colonoscopy, mammography) and preventive options (for example, preventive mastectomy), which may reduce cancer diagnoses in the family. 

Insurance Concerns:

In 2017, the Canadian government passed a law entitled the “Genetic Non-Discrimination Act.” It was upheld in the Supreme Court in 2020. This law prevents any person or organisation (including insurance companies) from requiring genetic test results for providing services, entering a contract or changing terms of an agreement. This means it is illegal for your insurance company to request your genetic results or require you to take a genetic test. https://laws-lois.justice.gc.ca/eng/acts/G-2.5/page-1.html

Getting a referral:

If you are a pancreatic cancer patient, your doctors may offer you genetic counselling and testing as part of the care that you will receive in the cancer clinic. This may be done as part of a research study. You can also ask your medical team for a medical genetics referral. If you are not a pancreatic cancer patient, but you are concerned about your family history of pancreatic cancer, you can request a medical genetics referral from your family physician. The medical genetics team will then use the family history information in the referral to determine if you need to be seen in clinic. Try your best to gather as much information about your family’s medical history in preparation for the appointment, including specific cancer diagnoses in your family, the age of each relative at diagnosis, and if any of your relatives have had genetic testing.

Direct-to-consumer genetic testing:

There are companies that offer genetic testing to the general public. Since the genetics associated with pancreatic cancer are complex, we recommend that you discuss genetic testing with your doctor before pursing direct-to-consumer testing.

References:

Greer, J. B. et al. 2009. Hereditary pancreatic cancer: A clinical perspective. Best Practice & Research Clinical Gastroenterology 23(2), pp. 159-170. https://doi.org/10.1016/j.bpg.2009.02.001

National Society of Genetic Counselors. Who are Genetic Counselors? About Genetic Counselors. https://www.nsgc.org/page/whoaregcs Accessed 16 Jul 2020.

NSGC.org


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