Appendix CancerLast Updated: July 28, 2011 This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/11 Overview
About the appendix 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 arises 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. Learn more about carcinoid tumors. 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 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. Find out more about basic cancer terms used in this section. Statistics
Primary appendix cancer (cancer that starts in the appendix) is uncommon, and statistics for appendix cancer are typically included as part of colorectal cancer data. It is estimated that about 1% of colorectal cancer cases in the United States are primary appendix cancer, affecting about 1,400 people each year. The overall five-year survival rate (percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases) of people with appendix cancer varies depending on several factors, including the type of tumor. 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: The National Cancer Institute and Sugarbaker, Paul. “New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?” Lancet Oncology. January 2006; 7(1):69-76. Risk Factors
A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors can 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. Symptoms and 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.
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. 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:
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. Learn more about what to expect when having common tests, procedures, and scans. After these diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is cancer, these results also help the doctor describe the cancer; this is called staging. Learn more about the first steps to take after a diagnosis of cancer. Staging
Staging is a way of describing a cancer, such as where it 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:
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 hasextended 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:
Stage IV: The cancer has spread to distant parts of the body, no matter the size of the tumor and 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;
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. Recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (re-staging) using the system above. 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 (2010) published by Springer-Verlag New York, www.cancerstaging.net. Treatment
This section outlines treatments that are the standard of care (the best proven 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 treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials and Current 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. 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.) 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. Learn more about making treatment decisions. Surgery Surgery is the removal of the tumor and surrounding tissue during an operation. It is the most common treatment for appendix cancer. Most often, appendix cancer is low-grade (see Staging) 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 tumor smaller than 1.5 centimeters (cm). In cases when appendix cancer is discovered unexpectedly after an appendectomy 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 tumor larger than 2 cm, 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 (an opening in the abdomen through which the bowel contents are emptied into a bag). Debulking surgery. For advanced appendix cancer, debulking (or cytoreduction) surgery may be performed. In this surgery, the doctor removes as much of the tumor “bulk” as possible, which could 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 any remaining cancer cells. In cases 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 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. 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 cancer surgery. Chemotherapy Chemotherapy is the use of drugs to kill 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, chemotherapy is most often used soon after surgery when cancer is found outside of the appendix region. 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 beyond 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 is delivered through the bloodstream to reach cancer cells throughout the body. This can be done using an intravenous (IV) tube (a tube inserted into a person’s vein). 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 are similar to those for colorectal cancer and can include fluorouracil (5-FU, Adrucil), leucovorin (Wellcovorin), capecitabine (Xeloda), irinotecan (Camptosar), oxaliplatin (Eloxatin), bevacizumab (Avastin), and cetuximab (Erbitux). The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away when treatment is finished. Learn more about 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 kill 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. In certain cases, a form of radiation called P32 may be recommended. In this procedure, radioactive phosphorus is dissolved in a liquid and placed inside the body after surgery through a tube inserted in the abdomen (see above). P32 delivers strong radiation therapy to a local area. Because the radioactivity disappears quickly (within a few hours), there is no need to remove the substance from the abdomen after treatment. 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 radiation therapy. Recurrent appendix cancer Once your treatment is complete and there is a remission (absence of cancer symptoms; also called “no evidence of disease” or NED), talk with your doctor about the possibility of the cancer returning. Many survivors feel worried or anxious that the cancer will come back. Learn more about coping with this fear. 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 therapies described above (such as surgery, chemotherapy, and radiation therapy) but 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. 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. 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 seeking 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. In addition to treatment to slow, stop, or eliminate the cancer (also called disease-directed treatment), an important part of cancer care is relieving a person’s symptoms and side effects. It includes supporting the patient with his or her physical, emotional, and social needs, an approach called palliative or supportive care. People often receive disease-directed therapy and treatment to ease symptoms at the same time. If disease-directed treatment is not successful, this may also be called advanced cancer. This diagnosis is stressful, and it may be difficult to discuss. 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. Learn more about advanced cancer care planning. Find out more about common terms used during cancer treatment. About Clinical Trials
Doctors and scientists are always looking for better ways to treat patients with appendix cancer. To make scientific advances, doctors create research studies involving people, called 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. 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, by participating in a clinical trial, they may receive no treatment by given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. 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. 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. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find clinical trials. For specific topics being studied for appendix cancer, learn more in the Current Research section. 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. 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 trials ends, and/or if the patient chooses to leave the clinical trial before it ends. Side Effects
Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects occur. Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and your overall health. Common side effects for each treatment option are described in detail within the Treatment section. Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health care team. Also, be sure to communicate with your doctor about side effects you experience during and after treatment. Care of a patient’s symptoms and side effects is an important part of a person’s overall treatment plan; this is called palliative or supportive care. It helps people with cancer at any stage of illness be as comfortable as possible. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Be sure to talk with your doctor 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 psychosocial (emotional and social) effects as well. For many patients, a diagnosis of appendix cancer is stressful and can bring difficult emotions. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. Learn more about the importance of addressing such needs, including concerns about managing the cost of your medical care. A side effect that occurs more than five years after treatment is called a late effect. Treatment of late effects is an important part of survivorship care. Learn more about late effects or long-term side effects by reading the After Treatment section or talking with your doctor. After Treatment
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. People treated for appendix cancer will generally need to follow up with an oncologist (a doctor who specializes in cancer), a surgeon, or an internal medicine specialist to monitor any symptoms of abdominal recurrence, such as pain, nausea, blood in the stool, severe bloating, and cramping. CT or MRI scans may be recommended as part of follow-up care. ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed. People recovering from appendix cancer are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about the next steps to take in survivorship, including making positive lifestyle changes. Find out more about common terms used after cancer treatment is complete. Current Research
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 new approach is called hyperthermic intraoperative peritoneal chemotherapy (HIPEC). This is similar to the hyperthermic peritoneal (local) chemotherapy described under Treatment, 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. Supportive 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. Learn more about common statistical terms used in cancer research. To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now. Questions to Ask the Doctor
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.
Patient Information Resources
In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease. View organizations that offer information on this specific type of cancer. |