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Small Intestine Cancer

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What is a small intestine cancer?

The gastrointestinal (digestive) system : The digestive system processes food for energy and rids your body of solid waste. After you chew and swallow your food, it enters the esophagus. This is a tube-shaped organ that carries food to your stomach. The esophagus joins thestomach just beneath the diaphragm (the breathing muscle under the lungs).

The stomach is a sack-like organ that holds swallowed food and begins the digestive process by secreting gastric juice. The food and gastric juices are mixed into a thick fluid, which is then emptied into the small intestine. The small intestine continues breaking down the food and absorbs most of the nutrients. Even though it is called the small intestine, it is actually the longest section of the gastrointestinal (GI) tract. The small intestine is about 15 to 20 feet long. Because of its length, in order to be contained in the abdomen, the small intestine has many loops.

The small intestine has 3 sections. The first section is the duodenum. It is short, only about 8 inches long. It is directly attached to the stomach and is where the stomach empties its contents. A short distance from where it attaches to the stomach, the pancreatic duct and bile duct attach to the duodenum. These discharge bile and pancreatic juices into the duodenum to further the digestive process. They attach at a site called the ampulla of Vater

The next 2 sections of the small intestine are called the jejunum and ileum. These parts of the intestine are where all the nutrients in food are absorbed into the bloodstream. They make up most of the length of the small intestine with the ileum being slightly longer. The duodenum goes into the jejunum. The ileum comes after the jejunum and ends when it empties into the large intestine (colon). The colon is a muscular tube about 4 to 5 feet long. The appendix is found near the place the ileum meets the colon. The colon continues to absorb water and mineral nutrients from the food matter and serves as a storage place for waste. The waste left after this process goes into the rectum. From there it passes out of your body through the anus.

Small intestine cancer : There are 4 major types of small intestinal cancer. Carcinoid tumors, gastrointestinal stromal tumors, and lymphomas make up about 60% to 70% of small intestine cancer. We have separate documents that talk about these 3 types. This document is about the 4th type of small intestinal cancer -- adenocarcinoma.

Adenocarcinomas make up about 30% to 40% of small intestine cancers. This type of cancer starts from the cells that line the intestine. Most experts think that cancer of the small intestine develops much like colorectal cancer. It first begins as a small benign outgrowth called a polyp. Over time, the polyp can change into a cancer. Most small intestinal cancers develop in the duodenum and the rest occur in the jejunum and ileum.

A major site of cancer in the duodenum is the ampulla of Vater. But because this area is closely associated with the pancreas, it is treated like pancreatic cancer and discussed in our separate document called Pancreatic Cancer

What are the risk factors for small intestine adenocarcinoma?

A risk factor is anything that changes your chance of getting a disease such as cancer. Different cancers have different risk factors. For example, exposing skin to strong sunlight is a risk factor for skin cancer. Smoking is a risk factor for many cancers. But risk factors don't tell us everything. Someone without any risk factors can develop cancer, and having a risk factor, or even several, does not mean that you will get the disease. Because small intestine adenocarcinoma is so uncommon, risk factors for this disease have been hard to study. Some of the known risk factors include:

Sex : Small intestinal adenocarcinoma occurs slightly more often in men than in women.

Age : Small intestinal adenocarcinoma becomes more common as people get older. The average age at diagnosis is about 60.

Smoking and alcohol use :  Some, but not all, studies have found an increased risk with either smoking or alcohol use.

Celiac disease : This disease is also known as celiac sprue and gluten-sensitive enteropathy. Gluten is a protein that is found in many types of grain, including wheat, rye, barley, and oats. In someone with celiac disease, eating gluten causes an immune reaction. The body makes antibodies that attack the lining of the intestines. This makes it hard to digest and absorb food, often leading to diarrhea and weight loss. People with celiac disease have an increased risk of small intestine cancers, including lymphoma and adenocarcinoma. Staying on a gluten-free diet seems to lower the risk of cancer in someone with this disease.

Colon cancer :  Survivors of colon cancer have an increased risk of getting cancer of the small intestine. This could be due to shared risk factors.

Crohns disease : Crohns disease is a condition in which the immune system attacks the gastrointestinal (GI) tract. This disease can affect any part of the GI tract, but it most often affects the lower part of the small intestine. People with this problem have a risk of small bowel adenocarcinoma that is about 28 times higher than normal. These cancers are most often seen in the ileum.

Diet :  One study has shown that a diet high in fiber may help lower the risk of small intestine cancer.

Inherited causes :  People with certain inherited conditions have a higher risk of small intestine adenocarcinoma.

Familial adenomatous polyposis (FAP) :  In this condition, many (even hundreds) of polyps develop in the colon and rectum. If the colon isn't removed, one or more of these polyps will become cancerous. Polyps in the stomach and the small intestine are also part of this syndrome, and they can lead to cancers in these areas. In FAP, most small intestine cancers are found in the duodenum. This condition is caused by an abnormal mutation (change) of the gene APC and is discussed more in our document called Colorectal Cancer.

Hereditary nonpolyposis colorectal cancer (HNPCC) : Another name for HNPCC is Lynch syndrome. In most cases, this disorder is caused by a defect in either the geneMLH1 or the gene MSH2, but at least 5 other genes can cause HNPCC: MLH3, MSH6, TGBR2, PMS1, and PMS2. An abnormal copy of any one of these genes reduces the body's ability to repair damage to its DNA. This results in an increased risk of cancer of the colon and small intestine, as well as a high risk of endometrial and ovarian cancer. People with this syndrome have up to a 4% chance of developing small intestine cancer. This condition is also discussed in our document called Colorectal Cancer.

Peutz-Jeghers syndrome (PJS) : People with this condition develop polyps in the stomach and intestines, as well as in other areas including the nose, the airways of the lungs, and the bladder. The polyps in the stomach and intestines are a special type calledhamartomas. They can cause problems like bleeding or blockage of the intestines. PJS can also cause dark freckle-like spots on the lips, inner cheeks and other areas. People with PJS have an increased risk of many types of cancer, including small intestine adenocarcinoma. This syndrome is caused by mutations in the gene STK1.

MUTYH-associated polyposis : People with this syndrome develop colon polyps which will become cancerous if the colon is not removed. They also can get polyps in the small intestine and have an increased risk of small intestine cancer. Other cancers that can occur in people with this syndrome include cancers of the skin, ovary, and bladder. This syndrome is caused by mutations in the gene MUTYH.

Cystic fibrosis (CF) : This condition causes severe lung problems. Often, in someone with CF, the pancreas cannot make the enzymes that break food down so that it can be absorbed. People with CF have an increased risk of adenocarcinoma of the ileum. The gene that causes CF is called CFTR. A child must have 2 abnormal copies of this gene (one from each parent) to get this disease.

Do we know what causes small intestine adenocarcinoma?

Very little is known about the causes of small intestine adenocarcinoma. In fact, many experts wonder why it is so rare. The small intestine is the longest structure in the gastrointestinal tract, yet it has only 2% or less of the adenocarcinomas.

As with other cancers, scientists have recognized some changes in the DNA of small intestine adenocarcinoma cells that are probably responsible for their increased growth and abnormal spread. Many of these tumors show specific genetic abnormalities. But the causes of these changes are not yet known.

One cause of these cancers is thought to be problems in repairing DNA, the large molecule that contains our genetic material. Certain genes control substances called enzymes that are responsible for repairing DNA when it makes mistakes in reproducing itself. Some have compared this to a spell checker on a computer. Without these spell checker enzymes, mistakes are not corrected and genetic mutations or changes are allowed to persist. These may cause the production of abnormal substances that lead to cancer formation.

A second theory is that mutations take place naturally with aging and that some of these will lead to cancer formation.

Can small intestine adenocarcinoma be prevented?

At this time, there is no known way to prevent most cases of small intestine adenocarcinoma. Since smoking may increase the risk of this cancer, not starting or quitting smoking may reduce the risk for this disease.

People with familial adenomatous polyposis (FAP) can have a very high risk of duodenal cancer. Doctors may suggest that these patients have surgery to remove the duodenum before cancer can develop. The procedure most often used is called a pancreaticoduodenectomy, a major operation that removes the duodenum, part of the pancreas, the gallbladder, the common bile duct, and part of the stomach. It is most often done in patients with FAP who have many polyps in the duodenum and so are at a high risk of getting duodenal cancer. This procedure is discussed in more detail in the "Surgery for small intestine adenocarcinoma" section of this document.

Can small intestine adenocarcinoma be found early?

If a person is genetically predisposed to develop small intestine adenocarcinoma, tests such as endoscopy can be done to find it early. These tests are discussed in detail in the next section. If someone with a genetic predisposition has symptoms of small intestinal adenocarcinoma, they should be tested immediately.

How is small intestine adenocarcinoma diagnosed?

If there is a reason to suspect you have a small intestinal cancer, the doctor will use one or more methods to find out if the disease is really present.

Signs and symptoms of small intestine adenocarcinoma : The symptoms of small bowel tumors are often vague. In one study, it took more than 6 months from the time of the first symptom until the diagnosis was made. The most common symptoms are:

Pain in the abdomen (belly)

Weight loss

Weakness and fatigue

Often, the first symptom is pain in the stomach area. This pain may start or get worse after you eat. As the tumor gets larger, it can start to block the passage of digested food. This can lead to increased pain -- it may be more intense and last longer. The tumor can cause a condition called obstruction. When this happens, the intestine is completely blocked and nothing can move through. This leads to pain with severe nausea and vomiting.

Rarely, a cancer will cause a hole in the wall of the intestine, letting the contents of the intestine spill into the abdominal cavity. This is condition is known as perforation. Symptoms of perforation include sudden severe pain, nausea, and vomiting.

Sometimes a tumor will start bleeding into the intestine. If the bleeding is slow, it could lead to anemia (a low red blood cell count). Symptoms of anemia include weakness and fatigue. If the bleeding is rapid, the stool can become black and tarry from digested blood and the patient may feel lightheaded or even pass out.

Medical history and physical exam : When a doctor takes your medical history, you will be asked questions about symptoms and risk factors you may have. The doctor will specifically ask about symptoms that could be caused by a mass in the intestines and examine you, concentrating on your abdomen looking for any swelling or sounds of the bowel trying to overcome a blockage.

Blood tests  : If your doctor suspects an intestine cancer, he or she will likely order some blood tests, such as:

A complete blood count (CBC) measures the cells in the blood, such as the red blood cells, white blood cells, and platelets. Small intestine cancer often causes low red blood cell count (anemia)

Blood chemistry tests to look for signs that a cancer has spread to the liver

Imaging tests  

Barium x-rays :  For these studies, a liquid containing barium (also called a contrast liquid) is swallowed to coat the lining of an area in the intestines or stomach, and then x-rays are taken. The barium helps outline abnormalities in the esophagus, stomach, and intestines, making them more visible. These x-rays are most often used to look at the upper or lower parts of the digestive system, and can help find tumors there. They are least helpful in finding small intestine tumors. Barium studies were used more often before endoscopy was available.

Upper GI series: This test, also known as a barium swallow, is a way to look at the upper part of the digestive tract. For this test, the patient drinks the barium solution and then x-rays are taken. The barium travels through the esophagus and stomach, and then into the first part of the small intestine. If the rest of the small intestine is to be looked at, this test may be called a small bowel follow-through. This test often gives good pictures of the first part of the small intestine (the duodenum), but the rest of the small intestine may be hard to see in detail.

Enteroclysis: This procedure gives more detailed pictures of the small intestine than the upper GI with small bowel follow-through. For this procedure, a tube is passed from the nose or mouth through the stomach and into the small intestine. Then, barium is sent through the tube directly into the small intestine. X-rays are taken as the contrast travels through the small bowel.

Barium enema: This is a way to look at the large intestine. Before this test, the bowel needs to be cleaned out. This is done by using strong laxatives and enemas the night before and the morning of the exam. For this test, the barium solution is given into the large intestine through the anus (like an enema). For better pictures, air is injected into the intestine through a tube. This is called air contrast. This procedure is meant to be used to look at the large intestine, but sometimes the last part of the small intestine can be seen as well.

Computed tomography :  A computed tomography (CT or CAT) scan is an x-ray procedure that makes detailed cross-sectional images of your body. Instead of taking one picture, like a conventional x-ray, a CT scanner takes many pictures as it rotates around you. A computer then combines these pictures into an image of a slice of your body. The machine takes pictures of multiple slices of the part of your body that is being studied.

A CT scanner has been described as a large donut, with a narrow table in the middle opening. You will need to lie still on the table while the scan is being done. CT scans take longer than regular x-rays, and you might feel a bit confined by the ring while the pictures are being taken.

CT scans are often used in people with abdominal pain to try to find the source of the problem. Although small intestine tumors may not be seen well by CT, these scans are good at showing some of the problems that these tumors can cause - like obstruction or perforation. CT scans are also helpful in staging cancer after it has been diagnosed. They can help tell if your cancer has spread into your lungs, liver, or other organs. They show the lymph nodes and organs where metastatic cancer might be present.

Before the test, you may be asked to drink 1 or 2 pints of a contrast liquid. This helps outline the intestine so that certain areas will not be mistaken for tumors. You may also get an IV (intravenous) line through which a different kind of contrast dye is injected. This helps better outline structures in your body.

The injection can cause some flushing (redness and warm feeling that may last hours to days). A few people are allergic to the dye and get hives. Rarely, more serious reactions like trouble breathing and low blood pressure can occur. Medicine can be given to prevent and treat allergic reactions. Be sure to tell the doctor if you are allergic to shellfish or have ever reacted to any contrast material used for x-rays.

CT scans are also used to guide a biopsy needle precisely into a suspected metastasis. For this procedure, called aCT-guided needle biopsy, the patient remains on the CT scanning table, while a radiologist moves a biopsy needle toward the location of the mass/tumor. CT scans are repeated until the doctors are confident that the needle is within the mass. A fine-needle biopsy sample (tiny fragment of tissue) or a core-needle biopsy sample (a thin cylinder of tissue about ½-inch long and less than 1/8-inch in diameter) is removed and looked at under a microscope.

Other tests

Upper endoscopy : This test uses an endoscope − a flexible lighted tube with a video camera on the end. The patient is usually first given medicine to make him or her sleepy. The endoscope goes in through the mouth, and then passes through the throat and the esophagus into the stomach and then into the first part of the small intestine. This allows the doctor to see clearly any masses in the lining of the digestive organs. If abnormalities are found, small pieces of tissue can be removed through the endoscope (biopsy). The tissue can be looked at under the microscope to find out if cancer is present and what kind of cancer it is.

Upper endoscopy (also called esophagogastroduodenoscopy or EGD) is used to look at the esophagus, stomach and duodenum (the first part of the small intestine). It is not a good way to look at the rest of the small intestine because the small intestine is so long and has many turns and loops. Newer methods, such as capsule endoscopy and double balloon endoscopy, are more helpful in looking at this area.

Capsule endoscopy : This procedure does not actually use an endoscope. Instead, the patient swallows a capsule (about the size of a large vitamin pill) that has a light and a very small camera. Like any other pill, the capsule goes through the stomach and into the small intestine. As it travels through the small intestine (usually over a period of about 8 hours), it takes thousands of pictures. The camera sends the images to a device that is worn around the person's waist while he or she goes on with normal daily activities. The pictures can then be downloaded onto a computer, where the doctor can look at them as a video. The capsule passes out of the body during a normal bowel movement and is flushed away.

Double balloon enteroscopy : Regular endoscopy cannot look very far into the small intestine because it is too long (20 feet) and has too many curves. This method gets around these problems by using a special endoscope that is made up of 2 tubes, one inside the other. First the inner tube, which is an endoscope, goes forward about a foot, and then a balloon at its end is inflated to anchor it. Then the outer tube goes forward to near the end of the inner tube and it is then anchored in place with a balloon. This process keeps being repeated over and over, letting the doctor see the intestine a foot at a time. An advantage of this over capsule endoscopy is that the doctor can take a biopsy of anything abnormal. This procedure is done after the patient is given drugs to make him or her sleepy.

Biopsy : A test finds a mass (tumor), but the only way to know if it is cancer is to do a biopsy. In a biopsy, a piece of the abnormal area is removed and examined under a microscope.

There are several ways to take a sample of an intestinal tumor. One way is through the endoscope. When a tumor is found, the doctor can use biopsy forceps (pincers or tongs) through the tube to take a small sample of the tumor. The specimen the doctor takes will be very small, but doctors can usually make an accurate diagnosis. Bleeding after a biopsy is a rare but potentially serious problem. If bleeding becomes a problem, doctors can sometimes inject drugs that constrict blood vessels through the endoscope into the tumor to stop the bleeding.

In some patients, surgery is needed to biopsy a tumor. This may be done if the tumor cannot be reached with an endoscope.

How is small intestine adenocarcinoma staged?

Staging is a process that tells the doctor how widespread your cancer may be when you are first diagnosed. It will show if the cancer has spread and how far. The treatment and outlook for small intestine cancer depends, to a large extent, on its stage. For early stage cancer, surgery may be all that is needed. For more advanced cancer, other treatments, such as chemotherapy or radiation therapy, may be required. Please be sure to ask your doctor to explain the stage of your cancer so that you can make the best choice about your treatment.

The staging system used is that of the American Joint Committee on Cancer (AJCC). It is also called the TNM system. Stages are described using the number 0 (zero) and the Roman numerals I through IV.

This staging system describes the spread of the cancer in relation to the layers of the wall of the small intestine. Because for most patients, this stage is unknown until after surgery, most doctors wait till then to decide on the cancer's stage. The stages described below are called pathologic stages. The pathologic stage is determined by the findings of the pathologist from looking at the cancer and other actual tissue that has been removed.

The AJCC/TNM System describes the extent of the primary Tumor (T), the absence or presence of metastasis to nearby lymph Nodes (N), and the absence or presence of distant Metastasis (M).

T categories for small intestine adenocarcinoma

T categories of small intestine cancer describe the extent of spread through the layers that form its wall.

These layers, from the inner to the outer, include the lining (mucosa), the fibrous tissue beneath this muscle layer (submucosa), a thick layer of muscle that contracts to force the contents of the intestines along (muscularis propria), and the thin outermost layers of connective tissue (subserosa and serosa) that cover the small intestine. The serosa is also known as the visceral peritoneum.

Tx: No description of the tumor's spread is possible because of incomplete information.

Tis: The cancer is only in the epithelium (the top layer of cells of the mucosa) − it has not grown into the deeper tissue layers. This is the earliest stage and is also known as carcinoma in situ (CIS).

T1: split into 2 groups

T1a: The cancer has grown from the top layer of cells of the mucosa and into the layer of connective tissue below (the lamina propria).

T1b: The cancer has grown through the mucosa and into the submucosa

T2: The cancer has grown through the mucosa and submucosa into the muscularis propria.

T3: The cancer has grown through the inner layers of the intestine wall (mucosa, submucosa, and muscularis propria) into the subserosa. It has not started to grow into any nearby organs or tissues.

T4: The cancer has grown through the entire wall of the small intestine (including the serosa). It may be growing into nearby tissues or organs.

N categories for small intestine adenocarcinoma

N categories indicate whether or not the cancer has spread to nearby lymph nodes.

Nx: No information about lymph node involvement is available (often because no lymph nodes have been removed).

N0: Lymph nodes near the tumor were checked and do not contain cancer.

N1: Cancer cells found in 1 to 3 nearby lymph nodes.

N2: Cancer cells are found in 4 or more nearby lymph nodes

M categories for small intestine adenocarcinoma

M categories indicate whether or not the cancer has spread to distant organs, such as the liver, lungs, or distant lymph nodes.

M0: No cancer has been found in other organs or tissues.

M1: Cancer has been found in other organs or tissues.

Stage grouping

The T, N, and M categories are combined (in a process called stage grouping) to determine the stage. The stage is expressed in Roman numerals from stage I (the least advanced stage) to stage IV (the most advanced stage). The following guide illustrates how TNM categories are grouped together into stages:

Stage 0: Tis, N0, M0

The cancer is in the earliest stage. It has not grown beyond the top layer of cells of the mucosa of the small intestine. This stage is also known as carcinoma in situ.

Stage I: T1 or T2, N0, M0

The cancer has grown from the top layer of cells of the mucosa and into deeper layers such as the lamina propria (T1a), the submucosa (T1b), or the muscularis propria (T2). It has not spread into nearby lymph nodes (N0) or distant sites (M0).

Stage IIA: T3 or T4, N0, M0

The cancer has grown through most of the wall of the small intestine and into the subserosa (T3 or T4). It has not yet spread to nearby lymph nodes (N0) or to distant sites (M0).

Stage IIB: T4, N0, M0

The cancer has grown through the wall of the intestine and into the serosa or into nearby tissues or organs (T4). It has not yet spread to nearby lymph nodes (N0) or to distant sites (M0).

Stage IIIA: Any T, N1, M0

The cancer has spread to 1 to 3 nearby lymph nodes (N1) but not to distant sites (M0).

Stage IIIB: Any T, N2, M0

The cancer has spread to 4 or more nearby lymph nodes (N2) but not to distant sites (M0).

Stage IV: Any T, Any N, M1

The cancer has spread to distant sites such as the liver, lung, peritoneum (the membrane lining the abdominal cavity), or ovary.

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