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Appendix Cancer - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Appendix Cancer. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About the appendix

The appendix is a pouch-like tube that is attached to the cecum, which is the first section of the large intestine or colon. The appendix averages 10 centimeters (about 4 inches) 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 cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. Another name for this type of cancer is appendiceal cancer. A benign tumor means the tumor can grow but will not spread.

Types of appendix tumors

There are different types 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 usually starts 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 50% 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. Learn more about carcinoid tumors.

  • Appendiceal mucoceles. Mucoceles are swellings or sacs from swelling of the appendix wall, typically filled with mucous. There is a range of benign to malignant conditions that can occur in the appendix to form a mucocele. Two of these conditions are mucinous cystadenomas and mucinous cystadenocarcinomas. Mucinous cystadenomas are benign and do not spread and they are similar to adenomatous polyps that can develop in the colon. When contained in the appendix, they can be completely removed with surgery. However, if the appendix ruptures, the cells may spread in the body cavity and continue to secrete jelly-like substance called mucin in the abdomen. The build-up of mucin can lead to abdominal pain, bloating, and changes in bowel function including bowel obstruction (blockage). Mucinous cystadenocarcinomas can have similar effects with mucin in the abdomen but they are malignant, meaning they can spread to other parts of the body.

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

  • Goblet cell carcinomas/Adenocarcinoids. Goblet cell carcinomas have features of both adenocarcinomas and carcinoid tumors. They are more aggressive than carcinoid tumors, and treatment is often similar to treatment for adenocarcinoma.

  • Paraganglioma. This 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.

The next section in this guide is Statistics and it helps explain how many people are diagnosed with this disease and general survival rates. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will find information about how many people are diagnosed with this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

Primary appendix cancer is cancer that starts in the appendix. This is uncommon, accounting for about 0.5% of all tumors that start in the GI tract.

The five-year survival rate is the percentage of people who survive at least five years after the cancer is found. The five-year survival rate for people with appendix cancer depends on many factors, including the stage of disease at time of diagnosis, type of appendiceal tumor (see Types of Appendix Tumors in the Overview section), and size.

In appendix carcinoid tumors, if the tumor size is less than 3 centimeters, without spreading to another part of the body, the five-year survival is about 100%. If the tumor is less than 3 centimeters and has spread to the regional lymph nodes, the five-year survival is 78%. If the tumor is larger than 3 centimeters, with or without spreading to other parts of the body, the five-year survival is also 78%.  If the cancer has spread to other parts of the body, the five-year survival rate is 32%.  Due to the rare nature of other types of appendix cancer, specific statistics are not available. Talk with your doctor about the factors related to your specific diagnosis.

Cancer survival statistics should be interpreted with caution. It is not possible to tell a person how long he or she will live with appendix cancer. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Source: Up to Date and the National Cancer Institute.

The next section in this guide is Risk Factors and it explains what factors may increase the chance of developing this disease. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of cancer. To see other pages, use the menu on the side of your screen.

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. The only consistent risk factor for various types of appendix cancer is increasing age. Appendix cancer is rare in children.

The next section in this guide is Symptoms and Signs and it explains what body changes or medical problems this disease can cause. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Appendix Cancer - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

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.

  • Appendicitis

  • Ascites, which is 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, which is the inability to have a child

If you are concerned about one or more of the symptoms or signs on this list, please talk with your doctor. Your doctor will ask how long and how often you’ve been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If cancer is diagnosed, relieving symptoms 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.

The next section in this guide is Diagnosis and it explains what tests may be needed to learn more about the cause of the symptoms. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose cancer and find out if it has spread to another part of the body, called metastasis. 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 spread.

This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition

  • Type of cancer suspected

  • Signs and 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. A pathologist then analyzes the sample(s).  A pathologist is 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, which is the surgical removal of the appendix. This is usually done for what is first 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 may be recommended to remove another margin of tissue around the area where the tumor began, depending on the type of tumor and size of tumor if it is a carcinoid tumor.

  • 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. A CT scan can also be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow.

  • Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow.

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

  • Radionuclide scanning (OctreoScan).  This test is used for carcinoid tumors and not other type of appendix cancers. 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.

After diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is appendix cancer, these results also help the doctor describe the cancer; this is called staging.

The next section in this guide is Stages and Grades, and it explains the system doctors use to describe the extent of the disease. Or, use the menu on the side of your screen to choose another section to continue reading this guide.

Appendix Cancer - Stages and Grades

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. In addition, you can read about how doctors evaluate and compare cancer cells to normal cells, called the grade. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out 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, which is the chance of recovery. There are different stage descriptions for different types of cancer.

TNM staging system

One tool that doctors use to describe the stage is the TNM system. Doctors use the results from diagnostic tests and scans to answer these questions:

  • Tumor (T): How large is the primary tumor? Where is it located?

  • Node (N): Has the tumor spread to the lymph nodes? If so, where and how many? 

  • Metastasis (M): Has the cancer metastasized to other parts of the body? If so, where and how much?

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), depending on the type of cancer. The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

Here are more details on each part of the TNM system for appendix cancer, listed first for carcinoid tumors and then for carcinomas:

Staging for carcinoid tumors of the appendix

Tumor (T)

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 (N)

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, which are lymph nodes near the appendix. All others are distant lymph nodes, which are 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.

Metastasis (M)

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

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 (T)

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, which is the next deepest layer of the appendix.

T2: The tumor has invaded the muscularis propria, which is 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, which is an area of fatty tissue next to the appendix that provides the blood supply for the appendix.

T4: The tumor has grown through the visceral peritoneum, which is the lining of abdominal cavity, or it 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 (N)

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, which are lymph nodes near the appendix. All others are distant lymph nodes, which are 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.

Metastasis (M)

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, which means the cancer has spread to organs or structures within the abdominal area.

M1b: There is nonperitoneal distant metastasis, which means the cancer has spread outside of the abdominal cavity.

Grade (G)

Doctors also describe this type of cancer by its grade (G), which describes how much cancer cells look like healthy cells when viewed under a microscope. The doctor compares the cancerous tissue with healthy tissue. Healthy tissue usually contains many different types of cells grouped together. If the cancer looks similar to healthy tissue and contains different cell groupings, it is called differentiated or a low-grade tumor. If the cancerous tissue looks very different from healthy tissue, it is called poorly differentiated or a high-grade tumor. The cancer’s grade may help the doctor predict how quickly the cancer will spread. In general, the lower the tumor’s grade, the better the 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, M, and G classifications.

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 has come back after treatment. If the cancer does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Seventh Edition published by Springer-Verlag New York, www.cancerstaging.net.

Information about the cancer’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Treatment Options. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will learn about the different ways doctors use to treat people with this type of cancer. To see other pages, use the menu on the side of your screen.

This section outlines treatments that are the standard of care (the best known treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new approach to treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Your doctor can help you review all treatment options. For more information, see the About Clinical Trials and Latest Research sections.

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team. Cancer care teams also include a variety of other health care professionals, including physician assistants, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

Listed below are descriptions of the most common treatment options for appendix cancer that is not a carcinoid tumor. Find treatment information for carcinoid tumors of the appendix, in another section of this website.

Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the patient’s preferences and overall health. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your doctor and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. It is the most common treatment for appendix cancer. Most often, appendix cancer is low-grade (see Stages and Grades) and, therefore, slow-growing. Often it can be successfully treated with surgery alone. A surgical oncologist is a doctor who specializes in treating cancer using surgery.

Types of surgeries for appendix cancer include:

  • Appendectomy. An appendectomy is the surgical removal of the appendix. It is usually the only treatment needed for an appendix carcinoid tumor smaller than 1.5 centimeters (cm).

    If appendix carcinoid cancer is discovered unexpectedly after an appendectomy that was performed for what was originally thought to have been appendicitis, a second operation to remove more tissue using surgical techniques (described below) is often recommended.

  • Hemicolectomy. For a carcinoid tumor larger than 2 cm or appendix cancers that are not carcinoid, a hemicolectomy may be recommended. This is the removal of a portion of the colon next to the appendix. Removal of nearby blood vessels and lymph nodes is often done at the same time. A right hemicolectomy is surgery performed on the right side of the colon. Even though a large amount of the large intestine is removed, the operation usually does not result in the need for a colostomy or stoma, which is an opening in the abdomen through which the bowel contents are emptied into a bag.

  • Debulking surgery. For later-stage appendix cancer, debulking (or cytoreduction) surgery may be performed. In this surgery, the doctor removes as much of the tumor “bulk” as possible, which can benefit the patient even though it will not remove every cancer cell from the body. Sometimes, debulking surgery will be followed with chemotherapy (see below) to destroy remaining cancer cells.

    When the tumor produces mucous, much of the bulk of the abnormal tissue often is not cancer but is due to accumulation of the mucous. The mucous looks like jelly, and this condition is often referred to as “jelly belly.” Removing the mucous from the abdomen can often relieve a patient’s symptoms of bloating.

  • Removal of the peritoneum. There is some controversy about the extent of surgery that is necessary in patients with slow-growing, low-grade appendix cancer that has spread beyond the colon to involve other areas of the abdomen. Some surgeons recommend aggressive surgery that includes the removal of the peritoneum (the lining of the abdomen) to remove as much of the cancer as possible. This type of surgery is also called a peritonectomy.

In patients with a very slow-growing tumor, such surgery can be effective in removing the majority of the cancer cells. This can benefit the patient by reducing the amount of cancer, even if it does not remove every cancer cell. However, it is a difficult operation that can have significant side effects. The doctor will consider many different factors, such as the patient’s age and overall health, before recommending this extensive surgery. Patients should talk with a specialist with expertise in this type of procedure beforehand.  

Learn more about the basics of cancer surgery.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. For appendix cancer that is not a carcinoid tumor, chemotherapy is most often used soon after surgery when cancer is found outside of the appendix region. It may also be given for a non-carcinoid appendix cancer after surgery to prevent the cancer from coming back, depending on the stage of disease. There are different types of chemotherapy, depending on how the drugs are delivered to the body:

  • Local/intraperitoneal chemotherapy. For local chemotherapy, the medication delivery is focused on one area or section of the body. This is the most common type of chemotherapy used in the treatment of appendix cancer. More specifically, it is called intraperitoneal chemotherapy, which is chemotherapy that is given directly into the abdominal cavity. Typically, the surgeon will try to remove as much of the tumor as possible (debulking surgery, see above) and then insert a tube in the abdomen through which chemotherapy can be given after the operation. In some cases, the chemotherapy is warmed above body temperature to increase its ability to penetrate the tissue that may be lined with tumor cells; this is called hyperthermic (or heated) intraperitoneal chemotherapy. Once chemotherapy is completed, the tube is removed, generally without the need for another operation.

  • Systemic chemotherapy. This type of chemotherapy gets into the bloodstream to reach cancer cells throughout the body. This can be done using an intravenous (IV) tube, which is a tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally). Some people may receive this type of chemotherapy in their doctor’s office or outpatient clinic; others may go to the hospital.

A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time. Specific drugs given in systemic chemotherapy for non-carcinoid appendix cancers are similar to those for colorectal cancer and can include fluorouracil (5-FU, Adrucil), leucovorin (Wellcovorin), capecitabine (Xeloda), irinotecan (Camptosar), oxaliplatin (Eloxatin), bevacizumab (Avastin), ziv-aflibercept (Zaltrap), ramucirumab (Cyramza), cetuximab (Erbitux), and panitumumab (Vectibix).

For chemotherapy for carcinoid tumors, see more information about carcinoid tumors in the Overview section.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away when treatment is finished.

Learn more about the basics of chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Radiation therapy is rarely used in the treatment of appendix cancer.  However, sometimes it may be used to treat a particular area when the cancer has spread (such as the bone) to help relieve symptoms (see below.) 

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.

Learn more about the basics of radiation therapy.

Getting care for symptoms and side effects 

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care is any treatment that focuses on reducing symptoms, improving quality of life, and supporting patients and their families. Any person, regardless of age or type and stage of cancer, may receive palliative care. It works best when palliative care is started as early as needed in the cancer treatment process. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional support and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, or radiation therapy. Talk with your doctor about the goals of each treatment in your treatment plan.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and palliative care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it can be addressed as quickly as possible. Learn more about palliative care

Metastatic appendix cancer

If cancer has spread to another location in the body, it is called metastatic cancer.

Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. Learn more about getting a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.

Your health care team may recommend a treatment plan that includes a combination of surgery, chemotherapy, and radiation therapy. Palliative care will also be important to help relieve symptoms and side effects.

For most patients, a diagnosis of metastatic cancer is very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called having “no evidence of disease” or NED. 

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the cancer returning. Understanding your risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If the cancer does return after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the treatment described above such as surgery, chemotherapy, and radiation therapy, but they may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer. Whichever treatment plan you choose, palliative care will be important for relieving symptoms and side effects.

People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

If treatment fails

Recovery from cancer is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.

This diagnosis is stressful, and advanced cancer is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Patients who have advanced cancer and who are expected to live less than six months may want to consider a type of palliative care called hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.

The next section in this guide is About Clinical Trials and it offers more information about research studies that are focused on finding better ways to care for people with cancer. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for patients with appendix cancer. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the U.S. Food and Drug Administration (FDA) was previously tested in clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Deciding to join a clinical trial

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating appendix cancer. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with appendix cancer.

Sometimes people have concerns that, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” However, placebos are usually combined with standard treatment in most cancer clinical trials. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

Patient safety and informed consent

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends.

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for appendix cancer, learn more in the Latest Research section.

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

In addition, this website offers free access to a video-based educational program about cancer clinical trials, located outside of this guide.

The next section in this guide is Latest Research and it explains areas of scientific research currently going on for this type of cancer. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of cancer and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about appendix cancer, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease.

However, research is hampered by the rare nature of the disease. Because appendix cancer is uncommon, appendix cancer-specific clinical trials may be challenging to find. Patients are encouraged to talk with their doctors about broader clinical trials that may be open to them, such as those studying gastrointestinal carcinoid tumors or colorectal cancer.

The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you.

  • Enhanced delivery of chemotherapy. Doctors are looking for different ways to deliver chemotherapy to the abdomen. One approach is called hyperthermic intraoperative peritoneal chemotherapy (HIPEC). This is similar to the hyperthermic peritoneal (local) chemotherapy described under Treatment Options, but the drugs are delivered directly to the open abdomen during debulking surgery, instead of after surgery.

  • Combination chemotherapy. Research is underway to determine the best combinations of different drugs that are the most effective for appendix cancer.

  • New medications to treat carcinoid tumors. While most chemotherapy treatments have been less effective for metastatic carcinoid tumors, there is a lot of research ongoing for new targeted therapies for carcinoid tumors as the biology of these types of tumors is becoming better understood. 

  • Palliative care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current appendix cancer treatments in order to improve patients’ comfort and quality of life.

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding appendix cancer, explore these related items that take you outside of this guide:

The next section in this guide is Coping with Side Effects and it offers some guidance in how to cope with the physical, emotional, and social changes that cancer and its treatment can bring. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

There are possible side effects for every cancer treatment, but patients don’t experience the same side effects when given the same treatments for many reasons. That can make it hard to predict exactly how you will feel during treatment. Common side effects from each treatment option for appendix cancer are described in detail within the Treatment Options section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your overall health.

Talking with your health care team about side effects

Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them.

And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with appendix cancer. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies, including concerns about managing the cost of your cancer care

During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the Follow-up Care section of this guide or talking with your doctor.

The next section in this guide is Follow-up Care and it explains the importance of check-ups after cancer treatment is finished. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Follow-Up Care

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will read about your medical care after cancer treatment is completed, and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

Care for people diagnosed with cancer doesn’t end when active treatment has finished. Your health care team will continue to check to make sure the cancer has not returned, manage any side effects, and monitor your overall health. This is called follow-up care.

After treatment for appendix cancer ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. CT or MRI scans may be recommended as part of follow-up care.

People treated for appendix cancer will generally need to follow up with an oncologist, a surgeon, or an internal medicine specialist to watch for a recurrence. Symptoms of a recurrence in the abdomen include pain, nausea, blood in the stool, severe bloating, and cramping.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will also ask specific questions about your health. Some people may have blood tests or imaging tests as part of regular follow-up care, but testing recommendations depend on several factors including the type and stage of cancer originally diagnosed and the types of treatment given.

Managing long-term and late side effects

Most people expect to experience side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. In addition, other side effects called late effects may develop months or even years afterwards. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on the type of cancer, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may also have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to ask about any concerns you have about your future physical or emotional health. ASCO offers forms to help create a treatment summary to keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed.

This is also a good time to decide who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the general care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your cancer care will lead your follow-up care, be sure to share your cancer treatment summary and survivorship care plan forms with him or her, as well as all future health care providers. Details about your cancer treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship and it describes how to cope with challenges in everyday life after a cancer diagnosis. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Survivorship

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will read about how to cope with challenges in everyday life after a cancer diagnosis. To see other pages, use the menu on the side of your screen.

What is survivorship?

The word survivorship means different things to different people. Two common definitions include:

  • Having no signs of cancer after finishing treatment.

  • The process of living with, through, and beyond cancer. According to this definition, cancer survivorship begins at diagnosis and includes people who continue to have treatment over the long term, to either reduce the risk of recurrence or to manage chronic disease.

In some ways, survivorship is one of the most complex aspects of the cancer experience because it is different for every person.

Survivors may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a cancer diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain of how to cope with everyday life.

Survivors may feel some stress when frequent visits to the health care team end following treatment. Often, relationships built with the cancer care team provide a sense of security during treatment, and people miss this source of support. This may be especially true as new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexuality and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing,

  • Thinking through solutions,

  • Asking for and allowing the support of others, and

  • Feeling comfortable with the course of action you choose.

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the center where you received treatment.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very important role in supporting a person diagnosed with cancer, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the cancer diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving in this article.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make positive lifestyle changes.

People recovering from appendix cancer are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

In addition, it is important to have recommended medical check-ups and tests (see Follow-up Care) to take care of your health. Cancer rehabilitation may also be recommended, and this could mean any of a wide range of services such as physical therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent and productive as possible.

Talk with your doctor to develop a survivorship care plan that is best for your needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note these links will take you to other sections of Cancer.Net:

  • ASCO Answers Cancer Survivorship Guide: This 44-page booklet (available as a PDF) helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about what comes next after finishing treatment.

  • Survivorship Resources: Cancer.Net offers an entire area of this website with resources to help survivors, including for survivors in different age groups.

The next section offers Questions to Ask the Doctor to help start conversations with your cancer care team. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team, to help you better understand your diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your cancer care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your care.

Questions to ask after getting a diagnosis

  • What type of appendix cancer do I have?

  • Can you explain my pathology report (laboratory test results) to me?

  • Is the cancer considered to be localized, regional, or advanced? What does this mean?

  • How often do you treat people with appendix cancer?

  • What are my treatment options?

  • What clinical trials are open to me? Where are they located, and how do I find out more about them?

  • What treatment plan do you recommend? Why?

  • What is the goal of each treatment? Is it to eliminate the cancer, help me feel better, or both?

  • What is my prognosis?

Questions to ask about having surgery

  • Can surgery be done to remove all of the cancer?

  • Can surgery be done to debulk the cancer? How will this help me?

  • How experienced is the surgeon with this type of operation?

  • What will my recovery be like after this operation?

  • Do you recommend chemotherapy or other treatment after surgery?

Questions to ask about having chemotherapy or radiation therapy

  • What type of treatment is recommended?

  • What is the goal of this treatment?

  • How long will it take to give this treatment?

  • What side effects can I expect during treatment?

  • What are the possible long-term effects of having this treatment?

  • What can be done to relieve the side effects?

Questions to ask about choosing a treatment and managing side effects

  • Who will be part of my health care team, and what does each member do?

  • Who will be coordinating my overall treatment?

  • What are the possible side effects of this treatment, both in the short term and the long term?

  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

  • Could this treatment affect my sex life? If so, how and for how long?

  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before cancer treatment begins?

Questions to ask about planning follow-up care

  • If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?

  • What follow-up tests will I need, and how often will I need them?

  • What support services are available to me? To my family?

  • Whom should I call for questions or problems?

The next section in this guide is Additional Resources, and it offers some more resources on this website beyond this guide that may be helpful to you. Or, use the menu on the side of your screen to choose another section to continue reading this guide.  

Appendix Cancer - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 07/2015

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Appendix Cancer. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Beyond this guide, here are a few links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Appendix Cancer. Use the menu on the side of your screen to select another section to continue reading this guide.