Appendix Cancer and Pseudomyxoma Peritonei
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Appendix Cancer and Pseudomyxoma Peritonei
Treatment for appendix cancer or PMP
You will be cared for by a multi-disciplinary team of health professionals during your treatment for appendix cancer or PMP. These may include a surgeon, medical oncologist (to prescribe and coordinate a course of systemic therapy which includes chemotherapy), radiation oncologist (to prescribe and coordinate a course of radiation therapy), nurse and allied health professionals such as a social worker, psychologist or counsellor,
dietitian, physiotherapist and occupational therapist.
Discussion with your doctor will help you decide on the best treatment for your cancer depending on:
- the type of cancer you have
- where it is in your body
- whether or not the cancer has spread (stage of disease)
- your age, fitness and general health
- your preferences.
The main treatments for appendix cancer and PMP are surgery and chemotherapy. These can be given alone or in combination and are an effective treatment with a little over 60% of patients receiving both cytoreductive surgery and HIPEC surviving beyond 10 years. PMP may not be treated straight away if the tumour is small and growing slowly; in this case it will be observed and monitored regularly, an approach known as active surveillance.
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.
PMP is usually treated with surgery: either cytoreductive surgery followed by chemotherapy (HIPEC) when aiming to cure PMP, or if the cancer cannot be treated effectively debulking surgery may be used instead to remove as much of the tumour as possible to reduce symptoms. Debulking surgery may be done again if the tumour grows back.
Types of surgery
Appendectomy – Surgery to remove the appendix. Often used for early stage appendiceal NETs.
Hemicolectomy – Surgery to remove a small part of the large bowel next to appendix; surrounding lymph nodes and blood vessels may also be removed during the procedure. Often used for appendiceal NETs at risk of spreading or appendix cancers that are not neuroendocrine.
Cytoreductive surgery (CRS or peritonectomy) – Surgery to remove all visible tumour from the abdominal cavity; part of the bowel and other organs including gallbladder, spleen, stomach and kidney may also be removed. In females the uterus, ovaries and fallopian tubes may be removed; in males the seminal vesicles may be severed. Often used for late-stage appendix cancer and PMP Chemotherapy.
If part of the bowel is removed during surgery, the surgeon will usually join it back together. If this isn’t possible, you may need a stoma where the end of the intestine is brought through an opening (the stoma) made in your abdomen and stitched onto the skin to allow faeces to be removed from the body and collected in a bag. The stoma may be temporary (where the operation is reversed later on) or permanent, depending on the amount of bowel that has been removed.
If you need a stoma, the surgeon will refer you to a stomal therapy nurse before surgery. These are nurses with special training in stoma care. They can answer your questions about adjusting to life with a stoma. For more information visit the Australian Association of Stomal Therapy Nurses at or call Cancer Council 13 11 20.
Chemotherapy (sometimes just called “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. This is because different drugs can destroy or shrink cancer cells in different ways.
Your treatment will depend on your situation and the type of cancer you have. It may also be used to help stop your cancer coming back after surgery. Your medical oncologist will discuss your options with you.
There are different types of chemotherapy used to treat appendix cancer and PMP:
Local chemotherapy – where the chemotherapy drugs are delivered directly to the cancer. When placed directly in the abdomen it is called intraperitoneal chemotherapy.
Systemic chemotherapy – 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.
Types of intraperitoneal chemotherapy
HIPEC (heated intraperitoneal chemotherapy) – The chemotherapy drug is heated to around 40°C (body temperature is about 37°C) to increase its effectiveness and placed directly in the abdomen to kill any tumour cells that remain after surgery or help control ascites (build-up of fluid); typically removed after 30–90 minutes.
EPIC (early post-operative intraperitoneal chemotherapy) – The chemotherapy drug is delivered to the abdomen the day after surgery using an access port (small plastic device); continued for several days. Usually used after HIPEC when able to be tolerated.
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.
A course of radiation therapy needs careful planning. During your first consultation you will meet with a radiation oncologist. At this session you will lie on an examination table and have a CT scan in the same position you will be placed in for treatment. The information from this session will be used by your specialist to work out the treatment area and how to deliver the right dose of radiation. Radiation therapists will then deliver the course of radiation therapy as set out in the treatment plan.
Radiation therapy does not hurt and is usually given in small doses over a period of time to minimise side effects.
Your doctor or nurse may suggest you take part in a clinical trial. Doctors run clinical trials to test new or modified treatments and ways of diagnosing disease to see if they are better than current methods. For example, if you join a randomised trial for a new treatment, you will be chosen at random to receive either the best existing treatment or the modified new treatment. Over the years, trials have improved treatments and led to better outcomes for people diagnosed with cancer.
For more information on appendiceal NET clinical trials contact:
Complementary therapies are designed to be used alongside conventional medical treatments (such as surgery, chemotherapy and radiation therapy) and can increase your sense of control, decrease stress and anxiety, manage fatigue and improve your mood.
Some complementary therapies and their clinically proven benefits are listed below:
acupuncture – reduces chemotherapy-induced nausea and vomiting; improves quality of life
aromatherapy – improves sleep and quality of life
art therapy, music therapy – reduce anxiety and stress; manage fatigue; aid expression of feelings
counselling, support groups – help reduce distress, anxiety and depression; improve quality of life
hypnotherapy – reduces pain, anxiety, nausea and vomiting
massage – improves quality of life; reduces anxiety, depression, pain and nausea
meditation, relaxation, mindfulness – reduce stress and anxiety; improve coping and quality of life
qi gong – reduces anxiety and fatigue; improves quality of life
spiritual practices – help reduce stress; instil peace; improve ability to manage challenges
tai chi – reduces anxiety and stress; improves strength, flexibility and quality of life
yoga – reduces anxiety and stress; improves general wellbeing and quality of life.
Some Australian cancer centres have developed “integrative oncology” services where evidence-based complementary therapies are combined with conventional treatments to create patient-centred cancer care that aims to improve both wellbeing and clinical outcomes.
Let your doctor know about any therapies you are using or thinking about trying, as some may not be safe or evidence-based.
Alternative therapies are therapies used instead of conventional medical treatments. These are unlikely to be scientifically tested and may prevent successful treatment of the cancer. Cancer Council does not recommend the use of alternative therapies as a cancer treatment.
If you have been diagnosed with appendix cancer or PMP, both the cancer and treatment will place extra demands on your body. Research suggests that eating well and exercising can benefit people during and after cancer treatment.
Eating well and being physically active can help you cope with some of the common side effects of cancer treatment, speed up recovery, improve sleep, and help improve your quality of life by giving you more energy, keeping your muscles strong, helping you maintain a healthy body weight and boosting your mood.
You can discuss individual nutrition and exercise plans with health professionals such as dietitians, exercise physiologists and physiotherapists.
All treatments can have side effects. The type of side effects that you may have will depend on the type of treatment and where in your body the cancer is. Some people have very few side effects and others have more. Your specialist team will discuss all possible side effects, both short and long-term (including those that have a late effect and may not start immediately), with you before your treatment begins.
One issue that is important to discuss before you undergo treatment is fertility, particularly if you want to have children in the future. This is especially the case if during your abdominal or pelvic surgery:
- your seminal vesicles are severed (in males)
- your uterus, ovaries and/or fallopian tubes are removed (in females).
Common side effects may include:
Surgery: general – Bleeding, damage to nearby tissue and organs (including nerves), drug reactions, pain, infection after surgery, blood clots, weak muscles (atrophy), lymphoedema.
Surgery: CRS or peritonectomy – Bowel leaks and slow return to normal bowel function, dehydration, loss of fertility (severing of seminal vesicles or removal of uterus, ovaries or fallopian tubes).
Chemotherapy – Fatigue, loss of appetite, nausea, bowel issues such as constipation or diarrhoea, hair loss, mouth sores, skin and nail problems, increased chance of infections, loss of fertility, early menopause.
Radiation therapy – Fatigue, loss of appetite, nausea, bowel issues such as diarrhoea, abdominal cramps and excess wind, bladder issues, hair loss, dry mouth, skin problems, lymphoedema, loss of fertility.
This information is reviewed by
This information was last reviewed February 2021 by the following expert content reviewers: John Henriksen, Consumer; Prof David Morris, Surgical Oncologist, St George Hospital, Sydney, NSW; Caitriona Nienaber, 13 11 20 Consultant, Cancer Council WA.