The appendix is an organ that forms part of the digestive system, situated near the junction of the large and small intestines. While its precise function is not entirely clear, it is believed to play a role in the immune system.
Appendix (or appendiceal) cancer develops when cells within the appendix begin to mutate and multiply uncontrollably, forming a tumour. These tumours can be either malignant (cancerous) or benign (non-cancerous). It is important to note that even benign tumours can occasionally undergo transformation and become malignant. Malignant tumours have the potential to spread to other parts of the body, whereas benign tumours typically remain confined to the appendix.
Appendix cancer is considered rare, with an estimated 3,000 diagnoses in indians each year. However, the number of reported cases has been increasing, particularly among patients under the age of 50. Approximately half of all cases are discovered incidentally during surgery for acute appendicitis. Other cases are identified when a mass is detected during a CT scan performed for an unrelated medical condition, or when the tumour has advanced to the point of causing noticeable swelling (distension) in the abdomen.
Given the rarity of appendix cancer, it is strongly advised that all patients diagnosed with an appendix tumour have their diagnostic tests formally reviewed and seek consultation from specialist doctors who have expertise in treating this specific type of cancer.
The prognosis for appendix cancer is dependent on various factors. These include the size and grade of the tumour (how abnormal the cells appear under a microscope), the depth of the tumour's spread within the appendix wall, and whether the tumour has spread beyond the appendix to other organs.
Appendix cancer is broadly categorised into two primary types:
Approximately half of all appendix cancers are neuroendocrine tumours (sometimes referred to as carcinoid tumours). These tumours originate from neuroendocrine cells, which are cells that receive signals from the nervous system and are responsible for releasing hormones. Many NETs are slow-growing and may not require immediate aggressive treatment. Tumours smaller than 2 centimetres are less likely to be malignant or to spread. Larger tumours generally require a more aggressive treatment approach.
These tumours begin in the epithelial lining of the appendix. They specifically affect the glandular cells, which are responsible for secreting bodily fluids. There are several categories of adenocarcinoma tumours:
This is the second most common type of appendix cancer. Mucinous tumours originate in the lining of the appendix and are characterised by their secretion of mucin, a thick, gelatinous protein found in mucus.
Also known as goblet cell adenocarcinomas, these affect both neuroendocrine cells and goblet cells within the appendix lining.
These tumours share similarities with colorectal cancer and often present with many of the same symptoms. They typically develop near the base of the appendix.
This is an aggressive form of tumour that can occasionally develop in the appendix. Symptoms may arise rapidly, necessitating immediate medical evaluation. Signet ring cell adenocarcinoma is often associated with poorer outcomes, though new treatment options are continually being researched and developed.
These are benign (non-cancerous) tumours that develop in the lining of the appendix. They are removed surgically. As long as the appendix remains intact, a mucinous cystadenoma is unlikely to spread to other organs.
Appendix cancer, particularly adenocarcinoma, can sometimes lead to a rare condition known as pseudomyxoma peritonei (PMP). PMP is characterised by the presence and growth of tumour cells in the abdomen that produce mucin (a component of mucus). While the tumour cells often begin in the appendix, PMP can also originate from the ovary or stomach. As the tumour grows and fills with mucus, it may rupture, causing mucus to spread throughout the peritoneal cavity (the space between the internal organs and the inner abdominal lining). This accumulation of mucinous tumour cells exerts pressure on other organs, potentially impairing digestive function and leading to malnutrition or an intestinal blockage.
While a definitive, direct cause of appendix cancer is not known, any factor that increases an individual's chance of developing the condition is considered a risk factor. These include:
Appendix cancer is most frequently diagnosed in individuals over 50, but is becoming more prevalent in those aged 40–49. It is very rare in children.
Women are more likely than men to develop NETs, including those in the appendix.
Conditions such as atrophic gastritis, pernicious anaemia, or Zollinger-Ellison syndrome, which interfere with the stomach's ability to produce acid, are considered risk factors.
Having a family history of multiple endocrine neoplasia type 1 (MEN1) syndrome (also called Wermer syndrome) is a risk factor.
Important Note: It is important to remember that not everyone with these risk factors will develop appendix cancer. If you have any of these risk factors, you should discuss them with your doctor.
Appendix cancer often does not present any symptoms until it is in an advanced stage and has spread to other parts of the body. When symptoms of appendix cancer are present, they can vary significantly from person to person. Possible symptoms may include:
Acute appendicitis (which is how most cases of appendix cancer are discovered during surgery)
Increase in abdominal size or girth, and bloating
Vague discomfort or pain in the lower right area of the abdomen
Pelvic discomfort
New hernias
Bowel obstruction
Changes in bowel habits
Ovarian masses (in women)
Acute or chronic abdominal pain
Important Note: These symptoms do not exclusively indicate appendix cancer and can be a sign of other health issues. It is therefore crucial to discuss any persistent symptoms with your doctor for a proper evaluation.
If you present with symptoms that may suggest appendix cancer, your doctor will perform a physical examination and take a detailed history of your health, lifestyle, and family medical background. However, since appendix cancer frequently remains asymptomatic in its early stages, it is often discovered incidentally during abdominal surgery for another condition, such as acute appendicitis, or during imaging tests like a CT scan for an unrelated problem.
If cancer is suspected or discovered, your doctor may use one or more of the following tests to confirm the diagnosis, determine the extent of its spread, and monitor the effectiveness of treatment.
While blood tests cannot provide a definitive diagnosis of appendix cancer, they are helpful for understanding your body's general state and for ruling out other conditions, such as an infection. Additionally, certain types of blood tests can detect proteins known as tumour markers, which are produced by cancer cells and may indicate the presence of cancer. This information assists doctors in deciding which further diagnostic tests are necessary.
If a patient has symptoms suggestive of appendix cancer, or if a tumour is found during abdominal surgery for another reason, doctors may remove a small sample of tissue (a biopsy) from the appendix for detailed testing. Examining this tissue under a microscope allows specialists to determine if cancer cells are present.
In some cases, appendix cancer is diagnosed only after an appendectomy (the surgical removal of the appendix) has been performed. If a patient is presenting with acute appendicitis symptoms, the doctor may perform a minimally invasive procedure, such as a laparoscopic appendectomy, to remove the appendix. The appendix tissue is then examined by a specialist. If tumour cells are found, doctors may recommend additional surgery to take a biopsy of the surrounding intestines to check for cancer spread.
Imaging tests are often used in conjunction with laboratory tests to help confirm a diagnosis. They are also vital for determining if the cancer has spread and for tracking its progression over time. Imaging tests may include:
A CT scan generates detailed, three-dimensional cross-sectional images of various internal body parts. Appendix cancer is sometimes found during a CT scan for a different condition. Doctors use the scan to measure the size of appendix tumours and to see if the cancer has spread to other organs, such as the intestines.
Using powerful magnets and radio waves, an MRI scan produces detailed, static images of the body. This helps doctors visualise the presence, size, and spread of tumours.
Following a diagnosis of appendix cancer, your doctor will determine the stage of the disease. Staging is a classification system that indicates how much cancer is present in the body and where it has spread at the time of diagnosis. This process is essential for the doctor to plan the most effective treatment strategy. The determined staging classification remains the same throughout the patient's journey, even if treatment is successful or if the cancer spreads later on.
As a leading cancer centre, SSCHRC manages a higher volume of patients with this complex type of cancer than most other hospitals. This experience provides an exceptional level of expertise and insight in its treatment.
Our highly respected surgeons are among the most recognised in the country, performing more appendix cancer procedures annually than many cancer surgeons perform in a lifetime. Furthermore, our medical oncology team is specialised in developing individualised treatment plans, utilising state-of-the-art systemic treatments and targeted therapies, in addition to clinical trials.
If you are diagnosed with appendix cancer, your doctor will discuss the optimal treatment options. The recommended approach is tailored and depends on several factors, including:
The specific type of tumour
Its location within the appendix
Whether the cancer has spread
Your overall health status
Your appendix cancer treatment will be customised to your particular needs. One or more of the following therapies may be recommended to treat the cancer itself or to help alleviate symptoms.
Surgery is the primary and main treatment for appendix cancer. The type of surgical procedure performed is dependent on the specific type of appendix cancer.
A procedure called a right hemicolectomy is often performed. This involves removing the appendix, the right portion of the colon, and the surrounding lymph nodes. If the tumour has not spread, surgery alone may be sufficient treatment.
Treatment for adenocarcinoma may involve a combination of the following:
Surgical removal of the tumour (known as cytoreduction or tumour debulking), combined with HIPEC, is generally the recommended course of action.
In certain cases, surgery may be combined with chemotherapy, which uses specific drugs to target and kill fast-growing tumour cells.
If appendix cancer has spread within the abdomen, the most effective approach typically involves cytoreductive (tumour debulking) surgery to remove the tumour and the accumulated mucin. This extensive surgery may involve removing parts of the intestine, gallbladder, ovaries, uterus, and the lining of the abdominal cavity.
Hyperthermic Intraperitoneal Chemotherapy (HIPEC), also referred to as heated chemotherapy, is carried out during the tumour debulking surgery. The abdominal cavity is filled with a chemotherapy drug that is heated to over 40 degrees Celsius (more than 104 degrees Fahrenheit). The abdomen is gently rocked back and forth for 90 minutes to ensure the drug reaches all areas of the abdominal cavity. This method allows the chemotherapy drugs to target tumour cells that the surgeon may not have been able to see.
Chemotherapy drugs are used to kill cancer cells, control their growth, or relieve symptoms related to the disease. Chemotherapy may involve a single drug or a combination of two or more drugs, depending on the specific type of cancer and its growth rate. The medical and surgical oncology care teams at SSCHRC will work collaboratively to determine the most appropriate type of chemotherapy for each individual patient.
SSCHRC offers a diverse selection of clinical trials to address the needs of our appendix cancer patients who may have limited standard treatment options. Your care team may recommend suitable clinical trials if appropriate.
Genetic profiling is also offered to patients to help determine which clinical trials may offer them the most benefit.
Our expert team is here to provide comprehensive care and support