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What is appendix cancer?

Appendix cancer occurs when cells in the appendix become abnormal and keep growing and form a mass or lump called a tumour.  

The type of cancer is defined by the particular cells that are affected and can be benign (noncancerous) or malignant (cancerous). The most common types of appendix cancer are: 

  • Mucinous adenocarcinoma – these start in epithelial cells that line the inside of the appendix. They can produce mucin (a jelly-like substance) and spread to other parts of the body. 
  • Neuroendocrine tumours (NETs) – these form in neuroendocrine cells inside the appendix. Appendiceal NETs are often found at the tip of the appendix. 
  • Goblet cell carcinoma (GCC) - these have features of both a NET and adenocarcinoma but behave more like an adenocarcinoma, which can be more aggressive. 
  • Colonic-type adenocarcinoma - these may behave like colon (large bowel) cancer and are often found at the base of the appendix. 

Appendix cancer is rare and the most common types are seen in people aged between 40 and 60 years.  



Appendix cancer symptoms

Appendix cancer may not cause symptoms in its early stages. However, some people may experience symptoms such as:  

  • appendicitis (lower right abdominal pain)

  • gradual increase in waist size

  • build-up of fluid in the abdomen  

  • bloating

  • changes in bowel habits

  • hernia

  • ovarian mass or lump.



Causes of appendix cancer

The causes of appendix cancer are not known. There are no clear risk factors and it does not appear to run in families. Increasing age, however, can increase the risk of appendix cancer. 



    Diagnosis of appendix cancer

    Appendix cancer is often found during abdominal surgery for a different condition or after an appendectomy (surgical removal of the appendix) for a suspected case of appendicitis. 

    If your doctor thinks that you may have appendix cancer, they will perform a physical examination and carry out certain tests. If the results suggest that you may have appendix cancer or PMP, your doctor will refer you to a specialist who will carry out more tests. These may include: 

    Blood tests

    Blood tests will include a full blood count to measure your white and red blood cells, your platelets and chemicals produced by cancer cells (tumour markers).  

    CT scan

    Special machines are used to scan and create pictures of the inside of your body. You may have an injection of dye into your veins before the scan which makes the pictures clearer.  

    During the scan you will lie on a table which moves in and out of the scanner.  A CT scan takes about 10-30 minutes.  

    MRI

    An MRI scan produces detailed cross-sectional pictures of your body and can show the extent of any tumours.  

    You will lie on a table which slides into a large metal tube that is open at both ends. An MRI scan takes about 30-90 minutes.  

    Ultrasound

    Soundwaves are used to create pictures of the inside of your body. You will be asked to lie down and a gel will be spread over the affected part of your body and then a small device (transducer) is moved over the area. The ultrasound takes about 15 minutes and is painless.  

    Diagnostic laparoscopy

    For a diagnostic laparoscopy, a think tube with a camera on the end (laparoscope) is inserted into the abdomen to view inside the cavity. The laparoscopy is performed under general anaesthetic.  

    Biopsy

    If your doctor sees any abnormal or unusual-looking areas they may remove a small sample of the tissue for closer examination. This is known as a biopsy. A pathologist will look at the sample under a microscope to check for signs of disease or cancer.  



    Treatment for appendix cancer

    The main treatments for appendix cancer are surgery and chemotherapy. These can be given alone or in combination.  

    Surgery

    Surgery is the main treatment for appendix cancer, especially for people with early-stage disease who are otherwise in good health. The type of operation depends on the location and stage of the tumour. 

    Chemotherapy

    Chemotherapy (sometimes referred to as “chemo”) is the use of drugs to kill or slow the growth of cancer cells. You may have one chemotherapy drug, or a combination of drugs.  

    There are different types of chemotherapy used to treat appendix cancer:  

    Local chemotherapy 

    This is where chemotherapy drugs are delivered directly to the cancer. When placed directly in the abdomen it is called intraperitoneal chemotherapy (see below).  

    Systemic chemotherapy  

    This is where the chemotherapy drugs enter the bloodstream and travel throughout the body to target rapidly dividing cancer cells in the organs and tissues. This type of chemotherapy is given through a drip into a vein (intravenously) or as a tablet that is swallowed. 

    Radiation therapy (radiotherapy)

    Radiation therapy (also known as radiotherapy) uses high energy rays to destroy cancer cells. It may be used for appendix cancer when it has spread to other parts of the body, such as the bone. Radiation therapy can shrink the cancer and relieve symptoms. 

    Palliative care

    In some cases of appendix cancer, your medical team may talk to you about palliative care. Palliative care aims to improve your quality of life by alleviating symptoms of cancer. 

    As well as slowing the spread of gall bladder cancer, palliative treatment can relieve pain and help manage other symptoms. Treatment may include radiotherapy, chemotherapy or other drug therapies. 

    Treatment Team

    Depending on your treatment, your treatment team may consist of a number of different health professionals, such as:
    • GP (General Practitioner)- looks after your general health and works with your specialists to coordinate treatment.
    • Surgeon- surgically removes tumours and performs some biopsies.
    • Medical oncologist- prescribes and coordinates the course of chemotherapy.
    • Radiation oncologist- prescribes and coordinates radiation therapy treatment.
    • Cancer nurses- assist with treatment and provide information and support throughout your treatment.
    • Dietitian- recommends an eating plan to follow while you are in treatment and recovery.
    • Other allied health professionals- such as social workers, pharmacists and counsellors..


    Screening for appendix cancer

    There is currently no national screening campaign available in Australia.  



    Preventing appendix cancer

    As the causes of appendix cancer are not known there is no preventative advice specific for this cancer.  



    Prognosis of appendix cancer

    It is not possible for a doctor to predict the exact course of a disease, as it will depend on each person's individual circumstances. However, your doctor may give you a prognosis - the likely outcome of the disease - based on the test results, the rate of tumour growth, as well as your age, fitness and medical history.  



    Sources

    Understanding Appendix Cancer and Pseudomyxoma Peritonei (PMP). Cancer Council Australia © 2021. Last medical review of the source fact sheet: February 2021. 

    This web-based resource was made possible by the Cancer Australia Supporting people with cancer Grant initiative, funded by the Australian Government.