The appendix
The appendix is a small tube that usually sits on the lower right side of the abdomen.
It hangs off the end of the caecum, which is a pouch at the start of the large bowel. The appendix does not have a clear function, but is thought to store gut bacteria and may play a role in the immune system to help prevent disease.
What are appendix cancer and Pseudomyxoma Peritonei (PMP)?
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 (non-cancerous) or malignant (cancerous). Malignant tumours have the potential to spread to other parts of the body through the blood stream or lymph vessels and form another tumour at a new site. This new tumour is known as secondary cancer or metastasis.
Types of appendix cancer
The most common types include:
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, including the peritoneum, which is a sheet of tissue that lines and protects organs in the abdomen (belly).
Neuroendocrine tumours (NETs) – These form in neuroendocrine cells inside the appendix. The neuroendocrine system is a network of glands and nerve cells that make hormones and release them into the bloodstream to help control normal body functions. 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.
Pseudomyxoma Peritonei (PMP)
Pseudomyxoma peritonei (PMP) is a rare tumour that grows slowly and causes a build-up of mucin (a jelly-like substance) in the abdomen and pelvis, giving rise to the name “jelly belly”. Several other diseases may also be associated with “jelly belly” including mucinous adenocarcinoma, or may resemble features of PMP including mucinous tumours in the bowel.
PMP often starts in the appendix but can also start in other organs such as the large bowel and ovary. While it doesn’t spread to other parts of the body, PMP can put pressure on important organs as it continues to grow and this may cause problems.
How common are appendix cancer and PMP?
Appendix cancer is rare with 0.12 cases per 1,000,000 people each year being reported for primary malignancies (cancer that first develops in the appendix). The most common types are seen in middle-aged people, with the typical age at diagnosis about 40 to 60 years. There is an almost equal risk for males and females for mucinous adenocarcinoma and GCC. Colonictype adenocarcinoma is diagnosed slightly more often in men and appendiceal NETs are diagnosed more often in women.
PMP is also rare with about 1 or 2 cases per 1,000,000 people each year. It is more likely to be diagnosed in people aged 40 years or over. Women may be diagnosed slightly more often and at an earlier stage than men, after a mass or lump is found in their ovary.
What are the symptoms and the risk factors?
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.
PMP is also difficult to detect, and symptoms may take a while to develop. Symptoms that some people may experience include:
- abdominal or pelvic pain
- gradual increase in waist size
- bloating
- changes in bowel habits
- hernia
- loss of appetite.
What are the risk factors?
The causes of appendix cancer and PMP are not known. There are no clear risk factors and neither appear to run in families. Increasing age, however, can increase the risk of appendix cancer.
How are appendix cancer and PMP diagnosed?
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. Similarly, PMP is often discovered when investigating a different condition.
If your doctor thinks that you may have appendix cancer or PMP, 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 including a full blood count to measure your white blood cells, red blood cells, platelets and tumour markers (chemicals produced by cancer cells).
CT (computerised tomography) or MRI (magnetic resonance imaging) scans
Special machines are used to scan and create pictures of the inside of your body. Before the scan you may have an injection of dye (called contrast) into one of your veins, which makes the pictures clearer. During the scan, you will need to lie still on an examination table.
For a CT scan the table moves in and out of the scanner which is large and round like a doughnut; the scan itself takes about 10 minutes.
For an MRI scan the table slides into a large metal tube that is open at both ends; the scan takes a little longer, about 30–90 minutes to perform. Both scans are painless.
Ultrasound scan
Soundwaves are used to create pictures of the inside of your body. For this scan, you will lie down and a gel will be spread over the affected part of your body. A small device called a transducer is moved over the area. The transducer sends out soundwaves that echo when they encounter something dense, like an organ or tumour. The ultrasound images are then projected onto a computer screen. An ultrasound is painless and takes about 15–20 minutes.
Diagnostic laparoscopy
A thin tube with a camera on the end (laparoscope) is inserted under sedation into the abdomen to view inside the cavity.
Biopsy
A biopsy is the removal of some tissue from the affected area for examination under a microscope. The biopsy may be done in one of two ways. In a core needle biopsy, a local anaesthetic is used to numb the area, then a thin needle is inserted into the tissue under ultrasound or CT guidance. An open or surgical biopsy is done under general anaesthesia. The surgeon will cut through the skin and use a tiny instrument with a light and camera (laparoscope) to view the affected area and use another instrument to take a tissue sample.
Finding a specialist
Rare Cancers Australia have a knowledgebase directory of health professionals and cancer services across Australia.
Pseudomyxoma Survivor have a directory of PMP surgeons and specialists in Australia.
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
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 or call Cancer Council 13 11 20.
Chemotherapy
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
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.
Clinical trials
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, visit Australian Cancer Trials or contact the Appendix Cancer/
Pseudomyxoma Peritonei Research Foundation (ACPMP).
For more information on appendiceal NET clinical trials contact:
Download our booklet ‘Understanding Clinical Trials and Research’
Complementary therapies
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.
Download our booklet ‘Understanding Complementary Therapies’
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.
Nutrition and exercise
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.
Download our booklet ‘Nutrition for People Living with Cancer’
Download our booklet ‘Exercise for People Living with Cancer’
Side effects of treatment
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).
Download our booklet ‘Fertility and Cancer’
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.
Life after treatment
Once your treatment has finished, you will have regular check-ups to confirm that the cancer hasn’t come back.
Ongoing surveillance for appendix cancer and PMP involves a schedule of ongoing scans and physical examinations. It’s important to let your doctor know immediately of any health problems between visits.
Some cancer centres work with patients to develop a “survivorship care plan” which usually includes a summary of your treatment, sets out a clear schedule for follow-up care, lists any symptoms to watch out for and possible long-term side effects, identifies any medical or psychosocial problems that may develop and suggests ways to adopt a healthy lifestyle going forward. Maintaining a healthy body weight, eating well and being physically active are all important.
If you don’t have a care plan, ask your specialist for a written summary of your cancer and treatment and make sure a copy is given to your GP and other health care providers.
What if the cancer returns?
For some people appendix cancer and PMP do come back after treatment, which is known as a recurrence. This is most likely to happen within the first five years after treatment. If the cancer does come back, treatment will depend on where the cancer has returned to in your body and may include a mix of surgery, chemotherapy and radiation therapy.
In some cases of advanced cancer, treatment will focus on managing any symptoms, such as pain, loss of appetite and improving your quality of life, without trying to cure the disease. This is called palliative treatment.
Palliative care can be provided in the home, in a hospital, in a palliative care unit or hospice, or in a residential aged care facility. Services vary, because palliative care is different in each state and territory.
When cancer is no longer responding to active treatment, it can be difficult to think about how and where you want to be cared for towards the end of life. However, it’s essential to talk about what you want with your family and health professionals, so they know what is important to you. Your palliative care team can support you in having these conversations.
Dealing with feelings of sadness
If you have continued feelings of sadness, have trouble getting up in the morning or have lost motivation to do things that previously gave you pleasure, you may be experiencing depression. This is quite common among people who have had cancer.
Talk to your GP, as counselling or medication—even for a short time—may help. Some people are able to get a Medicare rebate for sessions with a psychologist. Ask your doctor if you are eligible. Cancer Council SA operates a free cancer counselling program. Call Cancer Council 13 11 20 for more information.
For information about coping with depression and anxiety, visit Beyond Blue or call them on 1300 22 4636. For 24-hour crisis support, visit Lifeline or call 13 11 14.
