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appendix-cancer

Appendix Cancer

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What is Appendix Cancer?

The appendix is a pouch-like tube that is attached to the cecum (the first section of the large intestine or colon). The appendix averages 10 centimeters (cm) in length and is considered part of the gastrointestinal (GI) tract. Generally thought to have no significant function in the body, the appendix may be a part of the lymphatic, exocrine, or endocrine systems.

                                                                                                                              

Appendix cancer occurs when cells in the appendix become abnormal and multiply without control. These cells form a growth of tissue, called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). Another name for this type of cancer is appendiceal cancer.

Types of appendix tumors

There are a variety of tumors that can start in the appendix:

Carcinoid tumor. A carcinoid tumor starts in the hormone-producing cells that are normally present in small amounts in almost every organ in the body. A carcinoid tumor starts primarily in either the GI tract or lungs, but it also may occur in the pancreas, a man’s testicles, or a woman’s ovaries. An appendix carcinoid tumor most often occurs at the tip of the appendix. Approximately 66% of all appendix tumors are carcinoid tumors. This type of cancer usually causes no symptoms until it has spread to other organs and often goes unnoticed until it is found during an examination or procedure performed for another reason. An appendix carcinoid tumor that remains confined to the area where it started has a high chance of successful treatment with surgery. 

Mucinous cystadenocarcinoma. Mucinous cystadenocarcinoma is the most common non-carcinoid appendix tumor and accounts for about 20% of appendix cancer cases. This type of tumor produces a jelly-like substance called mucin that can fill the abdominal cavity and can cause abdominal pain, bloating, and changes in bowel function if the tumor breaks through the appendix or grows in the abdomen.

Colonic-type adenocarcinoma. Colonic-type adenocarcinoma accounts for about 10% of appendix tumors and usually occurs at the base of the appendix. This type of tumor looks and behaves like the most common type of colorectal cancer. It often goes unnoticed, and a diagnosis is frequently made during or after surgery for appendicitis (inflammation of the appendix that can cause abdominal pain or swelling, loss of appetite, nausea, vomiting, constipation or diarrhea, inability to pass gas, or a low fever that begins after other symptoms).

Signet-ring cell adenocarcinoma. Signet-ring cell adenocarcinoma (so called because, under the microscope, the cell looks like it has a signet ring inside it) is very rare and considered to be more aggressive and more difficult to treat than other types of adenocarcinomas. This type of tumor usually occurs in the stomach or colon, and it can cause appendicitis when it develops in the appendix.

Paraganglioma. Paraganglioma is a rare tumor that develops from cells of the paraganglia, a collection of cells that come from nerve tissue that persist in small deposits after fetal (pre-birth) development, and is found near the adrenal glands and some blood vessels and nerves. This type of tumor is usually considered benign and is often successfully treated with the complete surgical removal of the tumor. Paraganglioma is very rare outside of the head and neck region.

Symptoms & Signs

People with appendix cancer may experience the following symptoms or signs. Sometimes, people with appendix cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

Appendicitis

Ascites (fluid in the abdomen)

Bloating

Pain in the abdomen or pelvis area

Increased girth (size of the waistline), with or without a protrusion of the navel (bellybutton)

Changes in bowel function

Infertility (the inability to have a child)

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

Risk Factors

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

The cause of appendix cancer is unknown, and no avoidable risk factors have been identified. The following factor may raise a person’s risk of developing appendix cancer:

Age. For a carcinoid tumor of the appendix, the average age at diagnosis is approximately 40. Carcinoid tumors are rare in children.

Diagnosis

Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of cancer suspected

Severity of symptoms

Previous test results

In addition to a physical exam, the following tests may be used to diagnose appendix cancer:

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).

However, most often, appendix cancer is found unexpectedly during or after abdominal surgery. If cancer is suspected at the time of surgery, the doctor will remove a portion of the colon and surrounding tissue (called a margin) for examination. Often, a patient will have an appendectomy (surgical removal of the appendix) for what is thought to be appendicitis, and the cancer is diagnosed after the pathologist has processed and reviewed the tissue under the microscope. In that case, another surgery is usually recommended to remove another margin of tissue around the area where the tumor began.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture.

Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs.

Radionuclide scanning (OctreoScan). A small amount of a radioactive, hormone-like substance that is attracted to a carcinoid tumor is injected into a vein. A special camera is then used to show where the radioactive substance accumulates. This procedure is useful in detecting spread of a carcinoid tumor, especially to the liver.

Stages

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

One tool that doctors use to describe the stage is the TNM system. This system judges three factors: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

How large is the primary tumor, and where is it located? (Tumor, T)

Has the tumor spread to the lymph nodes? (Node, N)

Has the cancer metastasized to other parts of the body? (Metastasis, M)

The staging is different for carcinoid tumors in the appendix and carcinomas. Both staging systems are outlined below.

Staging for carcinoid tumors of the appendix

Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information is listed below.

TX: The primary tumor cannot be evaluated.

T0: There is no evidence of cancer in the appendix.

T1: The tumor is 2 centimeters (cm) or smaller.

T1a: The tumor is 1 cm or smaller.

T1b: The tumor is larger than 1 cm but no larger than 2 cm. 

T2: The tumor is larger than 2 cm but smaller than 4 cm, or it has extended into the large intestine.

T3: The tumor is larger than 4 cm or has extended into the small intestine.

T4: The tumor directly invades the abdominal wall or other nearby organs.

Node. The "N" in the TNM system stands for lymph nodes. The lymph nodes are tiny, bean-shaped organs that are located throughout the body that help the body fight infections as part of the body's immune system. There are regional lymph nodes (lymph nodes near the appendix). All others are distant lymph nodes (lymph nodes found in other parts of the body).

NX: The regional lymph nodes cannot be evaluated because of a lack of information.

N0: The cancer has not spread to the regional lymph nodes.

N1: The cancer has spread to the regional lymph nodes.

Distant metastasis. The "M" in the TNM system describes cancer that has spread to other parts of the body (such as the liver or lungs).

M0: The cancer has not spread to other parts of the body.

M1: The cancer has spread to other parts of the body.

Cancer stage grouping for carcinoid tumors of the appendix

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage I: The cancer is 2 cm or smaller and has not spread to the regional lymph nodes or to other parts of the body (T1, N0, M0).

Stage II: The cancer is larger than 2 cm and has or has not extended into the large or small intestine but has not spread to the regional lymph nodes or to other parts of the body (T2 or T3, N0, M0).

Stage III: Stage III cancer describes either of these situations:

The cancer has directly invaded the abdominal wall or has spread to other nearby organs but has not spread to the regional lymph nodes or to other parts of the body (T4, N0, M0).

The cancer is any size and may have spread to organs or structures near the appendix and has spread to the regional lymph nodes but not to distant parts of the body (T, N1, M0).

Stage IV: The cancer has spread to distant parts of the body, no matter the size of the tumor or whether it has spread to the regional lymph nodes (any T, any N, M1).

Staging for carcinomas of the appendix

Appendiceal carcinomas are also staged according to the TNM staging system.

Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information is listed below.

TX: The primary tumor cannot be evaluated.

T0: There is no evidence of cancer in the appendix.

Tis: This refers to carcinoma in situ (also called cancer in situ). Cancer cells are found only in the first layers lining the inside of the appendix.

T1: The tumor has invaded the submucosa (the next deepest layer of the appendix).

T2: The tumor has invaded the muscularis propria (the third layer of the appendix).

T3: The tumor has grown through the muscularis propria and into the subserosa (a thin layer of connective tissue) of the appendix or into the mesoappendix (an area of fatty tissue next to the appendix that provides the blood supply).

T4: The tumor has grown through the visceral peritoneum (the lining of abdominal cavity) or has invaded other organs.

T4a: The tumor has invaded the visceral peritoneum.

T4b: The tumor has invaded other organs or structures, such as the colon or rectum.

Node. The "N" in the TNM system stands for lymph nodes. The lymph nodes are tiny, bean-shaped organs that are located throughout the body that help the body fight infections as part of the body's immune system. There are regional lymph nodes (lymph nodes near the appendix). All others are distant lymph nodes (lymph nodes found in other parts of the body).

NX: The regional lymph nodes cannot be evaluated because of a lack of information.

N0: There is no regional lymph node metastasis.

N1: Cancer has spread to one to three regional lymph nodes.

N2: Cancer has spread to four or more regional lymph nodes.

Distant metastasis. The "M" in the TNM system describes cancer that has spread to other parts of the body (such as the liver or lungs).

MX: Distant metastasis cannot be evaluated.

M0: The cancer has not metastasized.

M1a: There is intraperitoneal metastasis (the cancer has spread to organs or structures within the abdominal area).

M1b: There is nonperitoneal distant metastasis (the cancer has spread outside of the abdominal cavity).

Tumor grade. Doctors may also use the term "grade," which describes how much the tumor appears like normal tissue under a microscope. The grade of a cancer can help the doctor predict how quickly the cancer might grow. In cancer that resembles normal tissue, doctors can clearly see different types of cells grouped together (called well differentiated). In a higher-grade cancer, cancer cells usually look less like normal cells, or "wilder" (called poorly differentiated or undifferentiated). In general, a patient with a more differentiated tumor has a lower grade and a better prognosis.

GX: The tumor grade cannot be identified.

G1: The tumor cells are well-differentiated.

G2: The tumor cells are moderately differentiated.

G3: The tumor cells are poorly differentiated.

G4: The tumor cells are undifferentiated.

Cancer stage grouping for carcinomas of the appendix

Doctors assign the stage of the cancer by combining the T, N, and M classifications. In describing Stage IV, doctors also consider the grade (G).

Stage 0: This refers to cancer in situ. The cancer is found in only one place and has not spread (Tis, N0, M0).

Stage I: The cancer has spread to inner layers of appendix tissue but has not spread to the regional lymph nodes or to other parts of the body (T1 or T2, N0, M0).

Stage IIA: The cancer has grown into the connective or fatty tissue next to the appendix but has not spread to the regional lymph nodes or to other parts of the body (T3, N0, M0).

Stage IIB: The cancer has grown through the lining of the appendix but has not spread to the regional lymph nodes or to other parts of the body (T4a, N0, M0).

Stage IIC: The tumor has grown into other organs, such as the colon or rectum, but has not spread to the regional lymph nodes or to other parts of the body (T4b, N0, M0).

Stage IIIA: The cancer has spread to inner layers of appendix tissue and to one to three regional lymph nodes but has not spread to other parts of the body (T1 or T2, N1, M0).

Stage IIIB: The cancer has grown into nearby tissue of the appendix or through the lining of the appendix and to one to three regional lymph nodes but has not spread to other areas of the body (T3 or T4, N1, M0).

Stage IIIC: This stage describes a cancer that has spread to four or more regional lymph nodes but not to other areas of the body (any T, N2, M0).

Stage IVA: This stage describes a cancer that has spread to other areas in the abdomen but not to the regional lymph nodes; the cancer cells are well differentiated (any T, N0, M1a, G1).

Stage IVB: Stage IVB describes any of these three situations;

The cancer has spread to other areas in the abdomen but not to the regional lymph nodes; the cells are moderately or poorly differentiated (any T, N0, M1a, G2 or G3).

The cancer has spread to other areas in the abdomen and to one to three regional lymph nodes; the cells may be any grade (any T, N1, M1a, any G).

The cancer has spread to other areas in the abdomen and to four or more regional lymph nodes; the cells may be any grade (any T, N2, M1a, any G).

Stage IVC: The cancer has spread outside the abdominal area to distant parts of the body, such as the lungs (any T, any N, M1b, any G).

Recurrent: For both carcinoid tumors and carcinomas, recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the appropriate system above.

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