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Pancreatic Cancer

Key Points

Pancreatic cancer (PC) is a relatively rare cancer of the pancreas gland.

• More than 46,000 people in the United States are diagnosed with PC every year and it is the fourth most common cancer killer. Over 39,000 people die annually from the disease.
• PC can be difficult to detect in its earliest stages, most patients are diagnosed after it has been growing for some time.
• A person with PC may or may not have symptoms, such as yellowed skin or eyes (jaundice), pale colored stool, back pain, unexplained weight loss and poor blood glucose control.
• PC is treated according to its disease stage. Treatments include chemotherapy, radiation, surgery, or in some cases, just supportive care.
• Not all abnormalities of the pancreas are cancer and a specific diagnosis is usually required.
• Diagnosis involves a physical exam, blood tests, taking special types of scans (such as a CT scan or MRI) and endoscopic studies, and in some cases a biopsy (sample) of the pancreas or a metastatic site.
• Taking care of patients with known or suspected PC often involves a variety of specialists. A variety of resources are available to patients and their families.

What is Pancreatic Cancer?
The pancreas is a gland deep in the abdomen, behind the stomach, that is part of the digestive and endocrine systems. It is situated near the liver, gallbladder (where bile is stored) and the beginning of the small intestine (duodenum). The pancreas makes juices that help with digestion (enzymes) as well as important hormones such as insulin that control the level of sugar in the bloodstream. PC is a condition in which cells grow abnormally and form a mass or tumor. Over time the mass continues to grow without heeding the body's usual checkpoints. Not all masses are cancerous, benign conditions such as chronic pancreatitis and autoimmune pancreatitis can simulate cancer of the pancreas. Sometimes, although rare, instead of forming a solid mass, PC can begin as a collection of fluid known as a cyst (most cysts however are not cancerous, they are benign).

About 46,000 people in the United States are diagnosed with PC every year and it is the fourth most common cancer killer. Over 39,000 people die annually from the disease. Little is known about the cause of most cases of PC. It is not contagious. There are a variety of types of PC and knowing which cells are involved is important. The most common cell type is known as adenocarcinoma. Other more rare forms of PC, which have unique treatments, include such types as neuroendocrine tumors and lymphoma. Patients being evaluated for pancreatic cancer typically meet several types of doctors, such as a gastroenterologist (a specialist for diseases of the digestive system), an oncologist (a doctor who specializes in cancer), a surgeon, and a radiation oncologist.

Symptoms
Unfortunately by the time most patients develop symptoms the cancer has become difficult to treat. Since the pancreas is near the drainage tubes for bile and digestive enzymes, a mass in the pancreas can block the flow of these substances into the small intestine. This can cause the skin and eyes to turn yellow (jaundice) and the stool to turn pale. It can also cause nausea, vomiting and itchy skin. PC can invade the nerves that run through the lower spine and cause back pain. If the digestive enzymes are not able to flow freely or if the pancreas cannot make enough insulin, patients can develop diarrhea or diabetes (high levels of sugar in the blood).

Who is at risk?
While PC is a relatively rare form of cancer, some people are more likely to develop it than others. Unfortunately most so called 'risk factors' cannot be changed, one main exception is smoking (smokers are up to three times more likely to develop PC than non-smokers). Other risk factors include age greater than 60, male gender, race (African Americans at higher risk), exposure to certain chemicals, or family history, having a close relative such as parent or sibling with PC.  There is some evidence that middle aged patients with newly diagnosed diabetes or with a long history of chronic pancreatitis are also at some increased risk. Having a relative with colon or ovarian cancer can also increase your risk of PC. Finally, while it is more common among men, women are also affected.

Diagnosis
The symptoms of PC can be confused with those of benign diseases such as arthritis (back pain), gallstones (jaundice) or chronic pancreatitis (pain, diarrhea, weight loss).

A variety of tests are used to detect and understand the extent or stage of the disease. It is important to detect PC at its earliest and most treatable stage. Precise staging helps determine what treatment course is best and what alternatives (such as participation in a clinical trial) might be appropriate. Because certain tests have special advantages and purposes, it is common for patients being evaluated for PC to undergo a series or combination of different tests.

After a physical examination and some basic blood tests (may include a special test known as CA 19-9), most patients get scanned with a CT (computerized tomography) or "CAT" scan of the abdomen. CT scans and MRI (magnetic resonance imaging) provide a birds-eye view of the pancreas and neighboring organs. They also allow doctors to see if the cancer has spread to areas outside the pancreas (metastases) or has wrapped itself around major blood vessels. Most patients with metastatic disease are not eligible for surgery.

A very important test, usually performed after a CT or MRI scan, is an endoscopic ultrasound (EUS). EUS allows specially trained doctors known as endoscopists to obtain highly detailed pictures of the pancreas and to get a biopsy (fine needle aspiration). This technique, which is performed under deep sedation, takes about one hour and patients can go home the same day it is performed in most cases. It uses a thin, flexible camera (combined with a tiny ultrasound device) that is passed gently through the mouth and into the stomach and small bowel. If the pain associated with PC cannot be managed by standard pain medicines, EUS can be used to inject an anesthetic directly into the nerves that may be responsible for the pain (celiac neurolysis).

Another test that may be required is known as ERCP (endoscopic retrograde cholangiopancreatography). During this test a small optical scope is used to inject a dye into the ducts that drain bile and the pancreatic juices. It also allows doctors to insert a small tube (stent) if needed to relieve a blockage. Both EUS and ERCP are performed by gastroenterologists that have had additional training in the technique. The American Society for Gastrointestinal Endoscopy (ASGE) maintains a listing of such specially trained doctors.

PTC (percutaneous transhepatic cholangiography) is performed by radiologists and involves injecting a dye through the skin and liver and into the bile ducts. It can tell if there is a blockage and, like ERCP, can be used to insert a drain to relieve a blockage.

Angiography can be performed using special intravenous injections and X-rays, CT scans or by MRI. It allows doctors to better understand if a cancer is wrapped around important blood vessels and determines how likely it is a tumor can be removed by surgery (resectability).

Laparoscopy is a surgical procedure in which tiny cameras are inserted into small incisions in the skin overlying the stomach. It is sometimes performed prior to more major surgery, such as removing some or all of the pancreas (see section on treatments, below), to determine resectability.

Screening for PC in relatives of affected people is not often done in the United States. However, families with many cases of PC or close relatives of people who developed PC at an early age should seek the advice of specialist such as a gastroenterologist or oncologist as there are specific genetic syndromes that may increase susceptibility for PC.

Treatment
Although there are many options, ranging from special medicines (chemotherapy) and radiation to surgical removal, cancer of the pancreas remains very difficult to treat. In most cases it is only those patients with very early stage disease who can be cured by surgery. Regardless of stage, there are many types of treatment that can help patients live longer and improve their quality of life. As mentioned earlier, the type or types or treatment available depend on the stage of the disease and the overall health of the patient.

Surgery is the most invasive, and most definitive, treatment for PC. The type of surgery considered depends on the size, location and stage of the cancer. A Whipple procedure treats cancer of the head of the pancreas (the widest part) and generally involves removing most of the pancreas and part of the small intestine, bile duct and stomach. A distal pancreatectomy involves removing the body and tail of the pancreas (preserving the head of the gland) as well as the spleen. A total pancreatectomy combines both procedures to remove the entire gland.

In some advanced cases of PC, sometimes the goal of surgery is not to cure but rather to relieve a blockage (obstruction) of the small intestine or bile ducts caused by a large mass. This procedure is known as a bypass.

Chemotherapy uses special medicines that target the cancer cells. One or a combination of such medicines is usually delivered by vein (intravenously) under the direction of a medical oncologist. Chemotherapy can sometimes kill normal cells as well and cause a variety of side effects. Oncologists are specially trained to help decide which drugs may be best and can help patients manage any side effects.

Radiation therapy (radiotherapy) uses high-energy X-rays to kill cancer cells. Radiation is directed by radiation oncologists and can be given alone or in combination with chemotherapy and surgery.

Clinical trials test the effectiveness of experimental treatments under closely monitored conditions. It is important to ask your doctor about all treatment options including those offered by the many ongoing clinical trials.

Finding a doctor/resources/getting a second opinion
ASGE website, ABMS website, NCI (http://cancer.gov/ or 1-800-4-CANCER), clinical trials (cancertrials.nci.nih.gov)