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Goblet Cell Carcinomas (GCC)

Goblet cell carcinomas (GCC) are rare neuroendocrine tumours that develop in the appendix. More specifically, they develop in goblet cells, which are responsible for mucus secretion and production. The mucus acts as a protective layer in the intestines.

Neuroendocrine cancers are a complex group of tumours that develop in the neuroendocrine system, which is responsible for regulating important bodily functions such as heart rate, blood pressure and metabolism. They most commonly develop in the gastro-intestinal tract, pancreas, and the lungs; however, they can develop anywhere in the body. These tumours develop from neuroendocrine cells, which are responsible for receiving signals from the nervous system and producing hormones and peptides (small proteins) in response. 

GCCs demonstrate a unique combination of both neuroendocrine cells and adenocarcinoma cells, which are cancerous cells that develop from mucus-producing cells. They tend to be more aggressive than classic neuroendocrine tumours, but are classified and staged as carcinomas of the appendix.

This type of cancer is diagnosed equally in males and females, and is generally diagnosed in people between the ages of 50-60. However, anyone can develop this disease. 

Treatment

If a GCC is detected, it will be staged and graded based on size, metastasis (whether the cancer has spread to other parts of the body) and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.

Cancers can be staged using the TNM staging system:

  • T (tumour) indicates the size and depth of the tumour.
  • N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
  • M (metastasis) indicates whether the cancer has spread to other parts of the body.

This system can also be used in combination with a numerical value, from stage 0-IV:

  • Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
  • Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and had not spread to lymph nodes. This stage is also known as early-stage cancer.
  • Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
  • Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also known as localised cancer.
  • Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.

Cancers can also be graded based on the rate of growth and how likely they are to spread:

  • Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
  • Grade II: cancer cells present as abnormal and grow faster than grade-I tumours. This is also known as an intermediate-grade tumour. 
  • Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour. 

Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate treatment option for you. 

Treatment is dependent on several factors, including location, stage of disease and overall health. 

Surgery 

Surgery is the most common treatment option for patients with GCCs. This option is most often used in patients who have a tumour that hasn’t metastasised (spread to other parts of the body). The procedure itself will vary depending on the location and size of the tumour, but usually involves removing the tumour and a margin of healthy tissue to help prevent recurrence.  In many cases, your surgeon may perform a total appendectomy, which is the removal of the appendix. This may be followed up with a right hemicolectomy, which is the removal of half of the large intestine/colon on the right side, near where the appendix is/was located.

In many cases, surgery is used in conjunction with chemotherapy or radiation therapy.  

Radiation therapy

Radiation therapy, or radiotherapy, uses controlled doses of radiation to damage or kill cancer cells. It can be administered internally or externally. This may be a satisfactory alternative if you are not a suitable candidate for surgery. This treatment can also be used in addition to surgery. In some cases, radiotherapy is recommended prior to surgery to shrink the tumour so it is easier to surgically remove. It can also be recommended after surgery to reduce the risk of cancer recurrence in the future. 

Chemotherapy 

Chemotherapy uses drugs to kill or slow the growth of cancerous cells, while minimising the damage to healthy cells. It is usually administered directly to the veins (intravenously), however in some cases in can be administered orally. You may receive a single chemotherapy drug, or a combination, depending on your individual factors. It is usually done in cycles of treatment sessions and periods of rest and can last several months. Chemotherapy can be recommended for patients prior to surgery in order to shrink the tumour, making it easier to surgically remove. In some cases, it can also be recommended after surgery to reduce the risk of cancer recurrence, or if your tumour is advanced and has metastasised.  

Targeted therapies

Targeted therapies, also known as molecular targeted therapies or biological therapies, are the use of drugs that target specific molecular features, or molecular targets, of cancer cells. These molecular targets may include gene mutations, mRNA (messenger RNA) levels, protein levels and/or enzyme activities that are essential for cancer growth. Using targeted drugs that attack these specific molecular features may block, kill or slow the growth of cancer cells, while minimising the damage to surrounding healthy cells. 

There are two main types of targeted therapy drugs that may be used:

  • Monoclonal antibodies, which are synthetic immune system antibodies that target specific proteins on the surface of cancer cells to inhibit its growth and survival.
  • Small molecular inhibitors, which are drugs that can block the production of certain enzymes and proteins required for growth from inside the cancer cell.

Ask your doctors about the availability of targeted therapies and whether you are suitable, as well as any benefits, risks and potential complications that may arise.  

Clinical trials 

Clinical trials are research studies performed to test new treatments. They present the opportunity for people, particularly those with rare or complex cancers, to receive very new treatments which are not yet available otherwise. While these studies have the potential to develop more treatment options, the risk of side effects can be high and are not always known. These trials might involve targeted therapies (to target only the cancer cells), hormonal therapies or immunotherapies.

Clinical trials are often conducted in phases:

  • Phase 0: these trials are used to determine how the body will react to experimental drugs. They are given in very small doses to very few people.
  • Phase I: these trials are used in slightly bigger groups (20-80 people) to determine the safety of the drug, as well as any potential side effects that may arise.
  • Phase II: these trials are used in larger groups (several hundred people) to further evaluate the safety of the drug, as well as whether it is working as it should be.
  • Phase III: these trials are used in groups of several hundred to several thousand people. They are used to monitor any adverse effects of the drug, as well as compare it to other conventional or experimental treatment methods.
  • Phase IV: these studies test the drug in the general population to determine any long-term adverse effects, as well as potential usage in treatment of other conditions. 

Ask your doctors about the availability of clinical trials and whether you are suitable, as well as any benefits, risks and potential complications that may arise.  

Palliative care 

Palliative care aims to improve the patient’s quality of life and alleviate symptoms, without trying to cure the disease. This option is beneficial for patients with advanced GCCs at any stage of treatment, not just towards the end of life. It can also be used in conjunction with other treatment options, such as surgery, radiotherapy, and chemotherapy, with the intention of relieving pain, alleviating symptoms, and meeting your physical, emotional, cultural, and spiritual needs. 

Risk factors

Because of how rare GCCs are, there has been limited research done into the risk factors for this disease. However, some studies have indicated that schistosomiasis (tropical parasite) may have a link to GCC.

Early symptoms

People who have GCC may experience some of the following symptoms:

  • Appendicitis.
  • Abdominal pain/distention (swelling).
  • Bowel obstruction (blockage of the bowels).
  • Abdominal lump.
  • Gastrointestinal bleeding.
  • Intussusception (part of the intestine slides into another part of the intestine).

Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.

Diagnosis/diagnosing

If your doctor suspects you have a GCC, they will order a range of diagnostic tests to confirm the diagnosis, and refer you to a specialist for treatment.
In many cases, GCC is diagnosed during an appendectomy to treat an appendicitis. 

Physical examination

Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities. 

Imaging & blood tests

The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), x-ray, and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a blood test may be taken to assess your overall health and help guide treatment decisions.

Biopsy

Once the location(s) of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. The tissue sample will then be analysed for cancer cells.

Prognosis (Certain factors affect the prognosis and treatment options)

While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on rate and depth of tumour growth, susceptibility to treatment, age, overall fitness, and medical history. Generally, early-stage GCCs have good prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. 

References

Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.