All About Pancreatic Cancer

Author: OncoLink Team
Content Contributor: Elizabeth Prechtel-Dunphy, DNP, CRNP, AOCN
Last Reviewed: November 6, 2019

What is the pancreas?

The pancreas is a pear-shaped gland, about six inches in length, located deep within the abdomen, between the stomach and the spine. It is referred to in 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 is responsible for making hormones, including insulin which helps regulate blood sugar levels and enzymes. These are then used by the bowel for the digestion of food. These enzymes are transported through ducts within the pancreas, emptied into the common bile duct, which carries the enzymes into the bowel.

What is pancreatic cancer?

Pancreatic cancer happens when cells in the pancreas begin to grow out of control. These "out of control" cancer cells then have the ability to spread to nearby lymph nodes and organs (such as the liver and lungs). When cancer spreads, it is called metastatic. About seventy percent of pancreatic cancers occur in the head of the pancreas, and most of these begin in the ducts that carry the enzymes. The vast majority of pancreatic cancers are of ductal origin and are referred to as pancreatic adenocarcinoma. There are other types of pancreatic cancer with different (non-ductal) cell origins, which make up a small minority of pancreatic cancers.

What causes pancreatic cancer and am I at risk?

It is estimated that there will be about 56,7670 new cases of pancreatic cancer diagnosed in the United States each year. Pancreatic cancers represent 3% of all cancer cases in the United States. The incidence of pancreatic cancer is highest between 60 and 80 years of age, and is only rarely seen in people under 40. It is slightly more common in men than women. 

Cigarette smokers are two times more likely to develop pancreatic cancer. Heavy alcohol users may also be at a higher risk of developing pancreatic cancer. It is seen more commonly in people who have diabetes and obese people, but this link is not yet well understood. Certain occupational exposures may also increase risk. These include chemists, coal, gas, and metal workers, and those employed in industries where pesticides are used more frequently. 

A person's risk is higher if his/her mother, father, or siblings have had the disease. A family history of breast or colon cancer also increases risk. This increased risk is due to inherited mutations in cancer-causing genes (changes that allow cancer to develop). The actual cause of this disease is not known but is thought to be a result of a combination of inherited genetic changes and changes caused by environmental exposures.

How can I prevent pancreatic cancer?

Unfortunately, no one really knows what causes the disease, so it is difficult to prevent. One important point is that the risk for smokers who quit does decrease; so giving up cigarettes is helpful. 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 screening recommendations currently available for pancreatic cancer if you are at average risk of getting the disease. For patients with 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, screening should be considered. This screening would begin 10 years earlier than the time when the disease is typically seen. For example, people with PJS tend to develop pancreatic cancer at an average age of 40, so screening would begin at age 30. Patients known to have these genetic syndromes should be followed by specialists that treat the syndrome. Studies that are used for screening include: CT scan, MRI, and EUS (endoscopic ultrasound), but these current methods are costly and researchers are looking for more cost-effective techniques.

Researchers have been able to discover the genetic changes present in cancer of the pancreas. These genes are detectable 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?

Unfortunately, the signs of early stage pancreatic cancer are vague, and often attributed to other problems by both patients and providers. More specific symptoms tend to develop after the tumor has advanced and grown to invade 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 located in the pancreas (head, body or tail). Newly developed diabetes can be a sign in some patients. This is caused by the cancerous pancreas' inability to produce insulin.

How is pancreatic cancer diagnosed?

When a healthcare provider suspects that a patient may have pancreatic cancer there are several tests that can be done to make a diagnosis. A high quality CT Scan (often a multiphase or pancreatic protocol CT) can detect a tumor in the pancreas, enlarged lymph nodes (which may indicate tumor involvement), tumors in the liver, or obstructions of the bile duct. Ultrasound can also be used. Ultrasound uses 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). During an EUS, a catheter is passed through the mouth down to the bowel and allows the provider to take a biopsy of the tumor. This helps to establish the size of the tumor, extension into adjacent structures, local and regional nodal and vascular involvement. An MRI can be used if a CT scan is inconclusive. PET/CT scans can provide additional imaging of the pancreas.

If a patient has jaundice, the 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 condition. Tests that can determine this are 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 inserted 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 inserted 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 examined by a pathologist.

Some patients with pancreatic cancer may have an elevated level of carbohydrate antigen 19-9 (CA 19-9), but this is not present in all cases and may be caused by other things. In patients who have an elevated level, it is useful in confirming a diagnosis in conjunction with radiology tests and for monitoring the disease during treatment. The level can be periodically checked during treatment to see if the cancer is stable or worsening. Other potential tumor markers associated with pancreatic cancer that may be evaluated include carcinoembryonic antigen (CEA).

How is pancreatic cancer staged?

The staging system used to describe pancreatic tumors is the "TNM system." The TNM system is used to describe many types of cancers. It has three components: T-describing the extent of the "primary" tumor (the tumor in the bladder itself); N-describing if there is cancer in the lymph nodes; M-describing the spread to other organs (metastases).

The staging system is very complex, and 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 a patient's cancer. The stage of cancer, or extent of disease, is based on information gathered through the various tests done as the diagnosis and work-up of the cancer is being performed. Practically speaking, pancreatic tumors are generally categorized as either resectable or unresectable, meaning whether or not they can be surgically removed. This depends on not only the size and spread, but whether or not critical structures (for example, major blood vessels) are involved.

How is pancreatic cancer treated?

Some patients have localized tumors that are able to be surgically removed (generally stages I & II), offering the best hope for a cure. This surgery, called a Whipple procedure or pancreaticoduodenectomy, is an extensive and complicated one, and recovery can be difficult for the patient. For this reason, it is important to only perform the procedure on patients with small, resectable tumors who are likely to benefit. Studies have found that the outcomes of the surgery are best when it is performed at a regional referral center that performs greater than 40 Whipple procedures per year. Even with curative resection, the tumor will come back without further treatment. These patients are treated with chemotherapy and radiation therapy after surgery (called adjuvant therapy) to provide the best chance of cure.

Some patients will present with tumors that cannot be surgically removed, but have not yet spread to distant organs (called locally advanced). For these patients, chemotherapy and radiation is standard treatment. Radiation is used to decrease the risk of the tumor recurring in the original area, while the chemotherapy is used to treat any stray cancer cells in the rest of the body. This combination decreases the risk of distant metastases. Chemotherapy agents are chosen for their ability to both kill cancer cells and make cells more sensitive to radiation (called radiosensitization). In some cases, the chemotherapy and radiation provide enough tumor shrinkage so that the patient 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 (Gemzar ®), in conjunction with radiation therapy. Other chemotherapy agents that may be used include: cisplatincapecitabine, albumin-bound paclitaxelirinotecan, docetaxel and oxaliplatin. These agents are often used in combination. 

Targeted therapies work by targeting a specific pathway necessary for the growth and development of the tumor cells. One targeted therapy being used in pancreatic cancer is erlotinib. Another therapy, pembrolizumab, has also been identified as a treatment in unresectable or metastatic pancreatic cancers with an MSI-H (metastatic microsatellite instability-high) or dMMR (mismatch repair deficient) tumor marker.

New therapies and combinations of existing therapies are allowing patients to live longer and have a better quality of life.

Patients with disease that has spread to other organs (metastasized) are usually treated with either 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.

Recent studies looking at the biologics and genetics of pancreatic cancer have focused on tumor markers, tumor metabolism, and tumor immunology.

Clinical Trials

There are clinical research trials for most types of cancer, and every stage of the disease. Clinical trials are designed to determine the value of specific treatments. Trials are often designed to treat a certain stage of cancer, either as the first form of treatment offered, or as an option for treatment after other treatments have failed to work. They can be used to evaluate medications or treatments to prevent cancer, detect it earlier, or help manage side effects. Clinical trials are extremely important in furthering our knowledge of 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 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 completion of therapy, patients are followed closely with CT scans and tumor marker levels (CA 19-9) for any sign of recurrence. Patients should follow up with their healthcare provider every 3 months for the first two years after completion of therapy and every 6-12 months after the first two years.

Fear of recurrence, 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 over 15 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, 8th Edition

Primary Tumor(T)

Description

TX

Primary tumor cannot be assessed

T0

No evidence of primary tumor

Tis

Carcinoma in situ

T1

Tumor ≤2 cm in greatest dimension 

T1a

Tumor < 0.5cm in greatest dimension

T1b

Tumor >0.5 cm and <1 cm in greatest dimension 

T1c

Tumor 1-2cm in greatest dimension

T2

Tumor > 2 cm and < 4cm in greatest dimension

T3

Tumor > 4cm in greatest dimension

T4

Tumor involves the celiac axis or the superior mesenteric artery and/or common hepatic artery, regardless of size

 

Regional Lymph Nodes (N)

Description

NX

Regional lymph nodes cannot be assessed

N0

No regional lymph node metastasis

N1

Metastasis in 1-3 regional lymph nodes

N2

Metastasis in 4 or more regional lymph nodes

 

Distant Metastasis (M)

Description

M0

No distant metastasis

M1

Distant metastasis

 

Stage 

T

N

M

Stage 0

T1s

N0

M0

Stage IA

T1

N0

M0

Stage IB

T2

N0

M0

Stage IIA

T3

N0

M0

Stage IIB

T1, T2, T3

N1

M0

Stage III

T1, T2, T3

N2

M0

T4

Any N

M0

Stage IV

Any T

Any N

M1

References

Alsamarrai A, Das, S.L., Windsor, J.Aa, Petrov, M.S. (2014). Factors that affect risk for pancreatic disease in the general population: a systematic review and meta-analysis of prospective cohort studies. Clinics Gastroenterol Hepatology, 12, 1635-164.

American Cancer Society. Pancreatic Cancer. https://www.cancer.org/cancer/pancreatic-cancer.html 

Anderson MA, Zolotarevsky E, Cooper KL, et al. (2012). Alcohol and tobacco lower the age of presentation in sporadic pancreatic cancer in a dose-dependent manner: a multicenter study. American Journal of Gastroenterology,107, 1730-1739.

Apte, M. V., & Wilson, J. S. (2016). Pancreatic cancer: A multipronged approach to pancreatic cancer treatment. Nature Reviews Gastroenterology & Hepatology13(7), 385-387.

Chuong, M. D., Springett, G. M., Freilich, J. M., Park, C. K., Weber, J. M., Mellon, E. A., ... & Shridhar, R. (2013). Stereotactic body radiation therapy for locally advanced and borderline resectable pancreatic cancer is effective and well tolerated. International Journal of Radiation Oncology* Biology* Physics86(3), 516-522.

De La Cruz, M. S., Young, A. P., & Ruffin, M. T. (2014). Diagnosis and management of pancreatic cancer. Am Fam Physician89(8), 626-632.

Garrido-Laguna, I., & Hidalgo, M. (2015). Pancreatic cancer: from state-of-the-art treatments to promising novel therapies. Nature Reviews Clinical Oncology12(6), 319-334.

Gresham, G. K., Wells, G. A., Gill, S., Cameron, C., & Jonker, D. J. (2014). Chemotherapy regimens for advanced pancreatic cancer: a systematic review and network meta-analysis. BMC Cancer14(1), 471.

Grote, T., & Gress, T. M. (2017). EUS and Its Role in Pancreatic Cancer. Pancreatic Cancer, 1-17.

Kamisawa, T., Wood, L. D., Itoi, T., & Takaori, K. (2016). Pancreatic cancer. The Lancet388(10039), 73-85.

Lin, Y., Egawa, N., & Kikuchi, S. (2016). Obesity and risk of pancreatic cancer: Epidemiologic evidence and perspective. Pancreatology16(4), S117.

Lucenteforte,E., La Vecchia, C., Silverman, D., et al.(2012).  Alcohol consumption and pancreatic cancer: a pooled analysis in the International Pancreatic Cancer Case-Control Consortium (PanC4). Annals of Oncology, 23, 374-382.

NCCN Clinical Practice Guidelines, Pancreatic Cancer, 2017, retrieved from https://www.nccn.org/professionals/physician_gls/pdf/pancreatic.pdf (log in required).

Neesse, A., Krug, S., Gress, T. M., Tuveson, D. A., & Michl, P. (2014). Emerging concepts in pancreatic cancer medicine: targeting the tumor stroma. OncoTargets and Therapy7, 33.

Paulson, A. S., Cao, H. S. T., Tempero, M. A., & Lowy, A. M. (2013). Therapeutic advances in pancreatic cancer. Gastroenterology144(6), 1316-1326.

Witvliet-van Nierop, J. E., Lochtenberg-Potjes, C. M., Wierdsma, N. J., Scheffer, H. J., Kazemier, G., Ottens-Oussoren, K., ... & de van der Schueren, M. A. E. (2017). Assessment of Nutritional Status, Digestion and Absorption, and Quality of Life in Patients with Locally Advanced Pancreatic Cancer. Gastroenterology Research and Practice2017.

Wolfgang, C. L., Herman, J. M., Laheru, D. A., Klein, A. P., Erdek, M. A., Fishman, E. K., & Hruban, R. H. (2013). Recent progress in pancreatic cancer. CA: A Cancer Journal for Clinicians63(5), 318-348.

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