Pancreatic Cancer, Exocrine

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Pancreatic Cancer, Exocrine

 

Definition

Exocrine pancreatic cancer is a disease in which cancerous cells originate within the tissues of the pancreas that produce digestive juices.

Description

The pancreas is a 6-8 in (15-20 cm) long, slipper-shaped gland located in the abdomen. It lies behind the stomach, within a loop formed by the small intestine. Other nearby organs include the gallbladder, spleen, and liver. The pancreas has a wide end (head), a narrow end (tail), and a middle section (body). A healthy pancreas is important for normal food digestion and also plays a critical role in the body's metabolic processes. The pancreas has two main functions, and each are performed by distinct types of tissue. The exocrine tissue makes up the vast majority of the gland and secretes fluids into the other organs of the digestive system. The endocrine tissue secretes hormones (like insulin) that are circulated in the bloodstream, and these substances control how the body stores and uses nutrients. The exocrine tissue of the pancreas, comprised mostly of acinar cells and ductal cells, produces pancreatic (digestive) juices. These juices contain several enzymes that help break down proteins and fatty foods. The exocrine pancreas forms an intricate system of channels or ducts, which are tubular structures that carry pancreatic juices to the small intestine where they are used for digestion.
Pancreatic tumors are classified as either exocrine or endocrine tumors depending on which type of tissue they arise from within the gland. Ninety-five percent of pancreatic cancers occur in the tissues of the exocrine pancreas. Ductal adenocarcinomas arise in the cells that line the ducts of the exocrine pancreas and account for 80% to 90% of all tumors of the pancreas. Unless specified, nearly all reports on pancreatic cancer refer to ductal adenocarcinomas. Less common types of pancreatic exocrine tumors include acinar cell carcinoma, cystic tumors that are typically benign but may become cancerous, and papillary tumors that grow within the pancreatic ducts. Pancreatoblastoma is a very rare disease that primarily affects young children. Two-thirds of pancreatic tumors occur in the head of the pancreas, and tumor growth in this area can lead to the obstruction of the nearby common bile duct that empties bile fluid into the small intestine. When bile cannot be passed into the intestine, patients may develop yellowing of the skin and eyes (jaundice) due to the buildup of bilirubin (a component of bile) in the bloodstream. Tumor blockage of bile or pancreatic ducts may also cause digestive problems since these fluids contain critical enzymes in the digestive process. Depending on their size, pancreatic tumors may cause abdominal pain by pressing on the surrounding nerves. Because of its location deep within the abdomen, pancreatic cancer often remains undetected until it has spread to other organs such as the liver or lung. Pancreatic cancer tends to rapidly spread to other organs, even when the primary (original) tumor is relatively small.
Though pancreatic cancer accounts for only 3% of all cancers, it is the fifth most frequent cause of cancer deaths. In 2001, an estimated 29,200 new cases of pancreatic cancer will be diagnosed in the United States. Pancreatic cancer is primarily a disease associated with advanced age, with 80% of cases occurring between the ages of 60 and 80. Men are almost twice as likely to develop this disease than women. Countries with the highest frequencies of pancreatic cancer include the United States, New Zealand, Western European nations, and Scandinavia. The lowest occurrences of the disease are reported in India, Kuwait and Singapore. African Americans have the highest incidence of pancreatic cancer of any ethnic group worldwide. Whether this difference is due to diet or environmental factors remains unclear.

Causes and symptoms

Although the exact cause for pancreatic cancer is not known, several risk factors have been shown to increase susceptibility to this particular cancer, the greatest of which is cigarette smoking. Approximately one-third of pancreatic cancer cases occur among smokers. People who have diabetes develop pancreatic cancer twice as often as non-diabetics. Numerous studies suggest that a family history of pancreatic cancer is another strong risk factor for developing the disease, particularly if two or more relatives in the immediate family have the disease. Other risk factors include chronic (long-term) inflammation of the pancreas (pancreatitis), diets high in fat, and occupational exposure to certain chemicals such as petroleum.
Pancreatic cancer often does not produce symptoms until it reaches an advanced stage. Patients may then present with the following signs and symptoms:
  • upper abdominal and/or back pain
  • jaundice
  • weight loss
  • loss of appetite
  • diarrhea
  • weakness
  • nausea
These symptoms may also be caused by other illnesses; therefore, it is important to consult a doctor for an accurate diagnosis.

Diagnosis

Pancreatic cancer is difficult to diagnose, especially in the absence of symptoms, and there is no current screening method for early detection. The most sophisticated techniques available often do not detect very small tumors that are localized (have not begun to spread). At advanced stages where patients show symptoms, a number of tests may be performed to confirm diagnosis and to assess the stage of the disease. Approximately half of all pancreatic cancers are metastatic (have spread to other sites) at the time of diagnosis.
The first step in diagnosing pancreatic cancer is a thorough medical history and complete physical examination. The abdomen will be palpated to check for fluid accumulation, lumps, or masses. If there are signs of jaundice, blood tests will be performed to rule out the possibility of liver diseases such as hepatitis. Urine and stool tests may be performed as well.
Non-invasive imaging tools such as computed tomography (CT) scans and magnetic resonance imaging (MRI) can be used to produce detailed pictures of the internal organs. CT is the tool most often used to diagnose pancreatic cancer, as it allows the doctor to determine if the tumor can be removed by surgery or not. It is also useful in staging a tumor by showing the extent to which the tumor has spread. During a CT scan, patients receive an intravenous injection of a contrast dye so the organs can be visualized more clearly. MRI may be performed instead of CT if a patient has an allergy to the CT contrast dye. In some cases where the tumor is impinging on blood vessels or nearby ducts, MRI may be used to generate an image of the pancreatic ducts.
If the doctor suspects pancreatic cancer and no visible masses are seen with a CT scan, a patient may undergo a combination of invasive tests to confirm the presence of a pancreatic tumor. Endoscopic ultrasound (EUS) involves the use of an ultrasound probe at the end of a long, flexible tube that is passed down the patient's throat and into the stomach. This instrument can detect a tumor mass through high frequency sound waves and echoes. EUS can be accompanied by fine needle aspiration (FNA), where a long needle, guided by the ultrasound, is inserted into the tumor mass in order to take a biopsy sample. Endoscopic retrograde cholangiopancreatography (ERCP) is a technique often used in patients with severe jaundice because it enables the doctor to relieve blockage of the pancreatic ducts. The doctor, guided by endoscopy and x rays, inserts a small metal or plastic stent into the duct to keep it open. During ERCP, a biopsy can be done by collecting cells from the pancreas with a small brush. The cells are then examined under the microscope by a pathologist, who determines the presence of any cancerous cells.
In some cases, a biopsy may be performed during a type of surgery called laparoscopy, which is done under general anesthesia. Doctors insert a small camera and instruments into the abdomen after a minor incision is made. Tissue samples are removed for examination under the microscope. This procedure allows a doctor to determine the extent to which the disease has spread and decide if the tumor can be removed by further surgery.
An angiography is a type of test that studies the blood vessels in and around the pancreas. This test may be done before surgery so that the doctor can determine the extent to which the tumor invades and interacts with the blood vessels within the pancreas. The test requires local anesthesia and a catheter is inserted into the patient's upper thigh. A dye is then injected into blood vessels that lead into the pancreas, and x rays are taken.
As of April 2001, doctors at major cancer research institutions such as Memorial Sloan-Kettering Cancer Center in New York are investigating CT angiography, an imaging technique that is less invasive than angiography alone. CT angiography is similar to a standard CT scan, but allows doctors to take a series of pictures of the blood vessels that support tumor growth. A dye is injected as in a CT scan (but at rapid intervals) and no catheter or sedation is required. A computer generates 3D images from the pictures that are taken, and the information is gathered by the surgical team who will develop an appropriate strategy if the patient's disease can be operated on.

Treatment

Staging

After cancer of the pancreas has been diagnosed, doctors typically use a TNM staging system to classify the tumor based on its size and the degree to which it has spread to other areas in the body. T indicates the size and local advancement of the primary tumor. Since cancers often invade the lymphatic system before spreading to other organs, regional lymph node involvement (N) is an important factor in staging. M indicates whether the tumor has metastasized (spread) to distant organs. In stage I, the tumor is localized to the pancreas and has not spread to surrounding lymph nodes or other organs. Stage II pancreatic cancer has spread to nearby organs such as the small intestine or bile duct, but not the surrounding lymph nodes. Stage III indicates lymph node involvement, whether the cancer has spread to nearby organs or not. Stage IVA pancreatic cancer has spread to organs near the pancreas such as the stomach, spleen, or colon. Stage IVB is a cancer that has spread to distant sites (liver, lung). If pancreatic cancer has been treated with success and then appears again in the pancreas or in other organs, it is referred to as recurrent disease.
Treatment of pancreatic cancer will depend on several factors, including the stage of the disease and the patient's age and overall health status. A combination of therapies is often employed in the treatment of this disease to improve the patient's chances for survival. Surgery is used whenever possible and is the only means by which cancer of the pancreas can be cured. However, less than 15% of pancreatic tumors can be removed by surgery. By the time the disease is diagnosed (usually at stage III), therapies such as radiation and chemotherapy or both are used in addition to surgery to relieve a patient's symptoms and enhance quality of life. For patients with metastatic disease, chemotherapy and radiation are used mainly as palliative (pain alleviating) treatments.

Surgery

Three types of surgery are used in the treatment of pancreatic cancer, depending on what section of the pancreas the tumor is located in. A Whipple procedure removes the head of the pancreas, part of the small intestine and some of the surrounding tissues. This procedure is most common since the majority of pancreatic cancers occur in the head of the organ. A total pancreatectomy removes the entire pancreas and the organs around it. Distal pancreatectomy removes only the body and tail of the pancreas. Chemotherapy and radiation may precede surgery (neoadjuvant therapy) or follow surgery (adjuvant therapy). Surgery is also used to relieve symptoms of pancreatic cancer by draining fluids or bypassing obstructions. Side effects from surgery can include pain, weakness, fatigue, and digestive problems. Some patients may develop diabetes or malabsorption as a result of partial or total removal of the pancreas.

Radiation therapy

Radiation therapy is sometimes used to shrink a tumor before surgery or to remove remaining cancer cells after surgery. Radiation may also be used to relieve pain or digestive problems caused by the tumor if it cannot be removed by surgery. External radiation therapy refers to radiation applied externally to the abdomen using a beam of high-energy x rays. High-dose intraoperative radiation therapy is sometimes used during surgery on tumors that have spread to nearby organs. Internal radiation therapy refers to the use of small radioactive seeds implanted in the tumor tissue. The seeds emit radiation over a period of time to kill tumor cells. Radiation treatment may cause side effects such as fatigue, tender or itchy skin, nausea, vomiting, and digestive problems.

Chemotherapy

Chemotherapeutic agents are powerful drugs that are used to kill cancer cells. They are classified according to the mechanism by which they induce cancer cell death. Multiple agents are often used to increase the chances of tumor cell death. Gemcitabine is the standard drug used to treat pancreatic cancers and can be used alone or in combination with other drugs, such as 5-flourouracil (5-FU). Other drugs are being tested in combination with gemcitabine in several ongoing clinical trials, specifically irinotecan (CPT-11) and oxaliplatin. Chemotherapy may be administered orally or intravenously in a series of doses over several weeks. During treatment, patients may experience fatigue, nausea, vomiting, hair loss, and mouth sores, depending on which drugs are used.

Biological treatments

Numerous vaccine treatments are being developed in an effort to stimulate the body's immune system into attacking cancer cells. This is also referred to as immunotherapy. Another type of biological treatment involves using a targeted monoclonal antibody to inhibit the growth of cancer cells. The antibody is thought to bind to and neutralize a protein that contributes to the growth of the cancer cells. Investigational treatments such as these may be considered by patients with metastatic disease who would like to participate in a clinical trial. Biological treatments typically cause flu-like symptoms (chills, fever, loss of appetite) during the treatment period.

Alternative treatment

Acupuncture or hypnotherapy may be used in addition to standard therapies to help relieve the pain associated with pancreatic cancer. Because of the poor prognosis associated with pancreatic cancer, some patients may try special diets with vitamin supplements, certain exercise programs, or unconventional treatments not yet approved by the FDA. Patients should always inform their doctors of any alternative treatments they are using as they could interfere with standard therapies. As of the year 2000, the National Cancer Institute (NCI) is funding phase III clinical trials of a controversial treatment for pancreatic cancer that involves the use of supplemental pancreatic enzymes (to digest cancerous cells) and coffee enemas (to stimulate the liver to detoxify the cancer). These theories remain unproven and the study is widely criticized in the medical community. It remains to been seen whether this method of treatment has any advantage over the standard chemotherapeutic regimen in prolonging patient survival or improving quality of life.

Prognosis

Unfortunately, cancer of the pancreas is often fatal, and median survival from diagnosis is less than six months, while the five-year survival rate is 4%. This is mainly due to the lack of screening methods available for early detection of the disease. Yet, even when localized tumors can be removed by surgery, patient survival after five years is only 10% to 15%. These statistics demonstrate the aggressive nature of most pancreatic cancers and their tendency to recur. Pancreatic cancers tend to be resistant to radiation and chemotherapy and these modes of treatment are mainly used to relieve pain and tumor burden.

Prevention

Although the exact cause of pancreatic cancer is not known, there are certain risk factors that may increase a person's chances of developing the disease. Quitting smoking will certainly reduce the risk for pancreatic cancer and many other cancers. The American Cancer Society recommends a diet rich in fruits, vegetables, and dietary fiber in order to reduce the risk of pancreatic cancer. According to the NCI, workers who are exposed to petroleum and other chemicals may be at greater risk for developing the disease and should follow their employer's safety precautions. People with a family history of pancreatic cancer are at greater risk than the general population, as a small percentage of pancreatic cancers are considered hereditary.

Key terms

Acinar cell carcinoma — A malignant tumor arising from the acinar cells of the pancreas.
Angiography — Diagnostic technique used to study blood vessels in a tumor.
Biopsy — Removal and microscopic examination of cells to determine whether they are cancerous.
Cancer vaccines — A treatment that uses the patient's immune system to attack cancer cells.
Chemotherapy — Drug treatment administered to kill cancerous cells.
Ductal adenocarcinoma — A malignant tumor arising from the duct cells within a gland.
Endoscopic retrograde cholangiopancreatography (ERCP) — Diagnostic technique used to obtain a biopsy. Also a surgical method of relieving biliary obstruction caused by a tumor.
Endoscopic ultrasonography (EUS) — Diagnostic imaging technique in which an ultrasound probe is inserted down a patient's throat to determine if a tumor is present.
Exocrine — Refers to glands which secrete their products through a duct.
Laparoscopic surgery — Minimally invasive surgery in which a camera and surgical instruments are inserted through a small incision.
Pancreatectomy — Partial or total surgical removal of the pancreas.
Radiation therapy — Use of radioisotopes to kill tumor cells. Applied externally through a beam of x rays, intraoperatively (during surgery), or deposited internally by implanting radioactive seeds in tumor tissue.
Whipple procedure — Surgical removal of the head of the pancreas, part of the small intestine, and some surrounding tissue.

Resources

Books

Teeley, Peter, and Philip Bashe. The Complete Cancer Survival Guide. New York: Doubleday, 2000.

Periodicals

Bornman, P. C., and I. J. Beckingham. "ABC of Diseases of Liver, Pancreas, and Biliary System. Pancreatic Tumours." British Medical Journal 322, no. 7288 (24 March 2001): 721-3.
Haut, E., A. Abbas, and A. Schuricht. "Pancreatic Cancer: The Role of the Primary Care Physican." Consultant 39, no. 12 (December 1999): 3329.
Parks, R. W., and O. J. Garden. "Ensuring Early Diagnosis in Pancreatic Cancer." Practitioner 244, no. 1609 (April 2000): 336-8, 340-1, 343.

Organizations

CancerNet. National Cancer Institute, 9000 Rockville Pike, Bldg. 31, Rm.10A16, Bethesda, Maryland, 20892. (800) 422-6237. http://wwwicic.nci.nih.gov.
Hirshberg Foundation for Pancreatic Cancer Research. 375 Homewood Rd., Los Angeles, CA 90049. (310) 472-6310. http://www.pancreatic.org.
National Pancreas Foundation. PO Box 935, Wexford, PA 15090-0935. http://www.pancreasfoundation.org.
Pancreatic Cancer Action Network. PO Box 1010, Torrance, CA 90505. (877) 272-6226. http://www.pancan.org.

Other

Johns Hopkins Medical Institutions. July 20, 2001 http://www.path.jhu.edu/pancreas.
Memorial Sloan-Kettering Cancer Center. Patient Information on Pancreatic Cancer. July 20, 2001. http://www.mskcc.org/patients_n_public/about_cancer_and_treatment/cancer_information_by_type/pancreatic_cancer/index.html.
University of Texas MD Anderson Cancer Center. Pancreatic Tumor Study Group. July 20, 2001. http://www.mdanderson.org/DEPARTMENTS/pancreatic/.
"What You Need To Know About Cancer of the Pancreas." National Cancer Institute. December 12, 2000. [cited July 20, 2001]. http://cancernet.nci.nih.gov/wyntk_pubs/pancreas.htm.
References in periodicals archive ?
The most common type by far, called exocrine pancreatic cancer, starts in the ducts that carry pancreatic juices.
What You Need to Know About Cancer of the Pancreas - an online publication about exocrine pancreatic cancer - at www.
Our work describes the incidences and morphologic aspects of pancreatitis and exocrine pancreatic cancer related to these DLCs, as observed in the 2-year toxicity and carcinogenicity studies.
Three different heredity scenarios can be considered: previously established genetic syndromes, hereditary chronic pancreatitis and familial exocrine pancreatic cancer (two or more pancreatic cancer cases in first-degree relatives) [2].
The Global Pancreatic Cancer Market 2014-2018 research report says based on the initiation, pancreatic cancer can be classified into exocrine pancreatic cancer and endocrine pancreatic cancer.