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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.
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:
This system can also be used in combination with a numerical value, from stage 0-IV:
Cancers can also be graded based on the rate of growth and how likely they are to spread:
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.
Treatment options for GCCs may include:
For more information on treatment options, please refer to the Rare Cancers Australia Treatment Options page.
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.
People who have GCC may experience some of the following symptoms:
Not everyone with the symptoms above will have cancer, but see your general practitioner (GP) if you are concerned.
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, GCCs are diagnosed during an appendectomy to treat an appendicitis.
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.
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.
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.
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.
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.
Page last updated: 26/04/2023