Goblet Cell Carcinoma of the Appendix (GCC)
The appendix can found near the junction where the large and small bowel meet. It is a small thin tube, approximately 4 inches long. Normally the appendix sits in the lower right abdomen. It has no clearly identified function in humans.
However, Goblet Cell Carcinoma (GCC) is distinct from Neuroendocrine Cancer of the Appendix by its appearance under a microscope and behaviour (growth rate). Because of this, GCC of the Appendix should be considered as a completely different entity to Neuroendocrine Cancer of the Appendix : they may have neuroendocrine features but also display characteristics of adenocarcinoma.
In Goblet Cell Carcinoma of the Appendix metastases (disease spread) are found in approximately 10% of all cases (most commonly to peritoneum, omentum, ovaries and / or liver).
Causes and / or risk factors for Goblet Cell Carcinoma (GCC)
We do not know exactly what causes Neuroendocrine Cancer – however it is important to follow advice in leading a healthy lifestyle: eat healthily, exercise and avoid smoking and too much alcohol.
Most Neuroendocrine Cancers do not run in families; however, a number of rare conditions may increase the risk of them developing. Therefore, if other members of the family have been diagnosed with cancer, or have a known genetic condition, it is important that you tell your specialist team about not only your personal medical history, but also any family medical illnesses or conditions.
We recommend speaking to your GP, and specialist team, if there is a strong family history of cancer – as genetic testing may be available for certain types of cancer and knowing your risk may help in planning care and managing future health.
GCC is rare – and is not associated with any known genetic alterations. However, if you do have a strong family history of a particular type of cancer – knowing this information may help your specialist team in planning your care and ensuring follow up is appropriate for you.
Symptoms that may or may not include Neuroendocrine Cancer associated syndromes
(Syndrome is where 2 or more related symptoms occur).
Goblet Cell Carcinoma (GCC) of the Appendix is uncommon and usually only discovered during tests or treatments for other conditions, that is, most are diagnosed following incidental findings after appendicectomy or small bowel resection (carried out for abdominal pain and right iliac fossa mass). Therefore symptoms, if they occur, may be the same as for suspected appendicitis, small bowel obstruction and /or ovarian conditions (e.g. bloating, wind, pain).
They are rarely associated with Neuroendocrine Cancer associated syndromes.
Other, rarer symptoms, including Paraneoplastic syndrome and oncological emergencies, (a specific set of health concerns that can occur in any cancer), such as raised calcium levels (Hypercalcaemia), may occur.
Further information about Neuroendocrine Cancer associated and Paraneoplastic Syndromes – including Oncological emergencies – can be found here.
Tests that may be used for the diagnosis and / or monitoring of GCC:
Blood and / or urine:
Full blood count
Liver and kidney function
CEA, Ca19-9, Ca-125.
Chromogranin A is rarely useful
Urinary or serum 5HiAA (serotonin).
CT chest, abdomen & pelvis and / or CT chest and MRI abdomen & pelvis
Octreotide (SPECT) or Gallium-Dotatate PET/CT is not routinely recommended.
Pathology (what can be seen through special tests under a microscope):
Differentiation and cellular morphology
Use of Tang criteria may be helpful – in terms of stratifying risk of recurrence
The key aim of treatment, should be to help you have the best possible care and quality of life – by ensuring access to appropriate treatment, management of symptoms and addressing what’s most important to you
Treatment options will depend on the type, position and size of your cancer – and whether (and to where) it has spread.
It will also depend on whether you have any other health concerns and / or illnesses and your general health and fitness.
One or more of the approaches below may be suggested:
- Control of your disease, by slowing or stopping further growth and / or spread
- Palliation, or easing, of any symptoms you may be experiencing.
Monitoring through clinic review, bloods and scans, can be used to assess how well treatment is working or in periods between treatments (which may be months/years).
As not everyone will need to be on treatment – surveillance can be used to check your cancer and general health for any signs of change that may mean that a treatment might need to be considered. All treatments have possible side-effects, therefore, it is important to know when treatment may be helpful for you or not.
Diagnosis may not occur until after an appendicectomy has been performed – further surgery may be required following full assessment of the removed appendix and surrounding tissue.
- Completion surgery may include a Right hemi-colectomy (removal of the ascending colon) +/- bilateral oophorectomy (removal of both ovaries) in female patients).
- If diagnosis is made before surgery occurs then R hemi-colectomy +/- bilateral oophorectomy + appendicectomy may be required and offered.
In advanced disease (where there is evidence of spread within the abdomen) referral to a Peritoneal Cancer specialist centre for consideration of Cytoreductive Surgery +/- HIPEC is recommended.
HIPEC is hyperthermic intra-peritoneal chemotherapy. It is considered in patients where there is a suspicion or evidence that cancer that has spread to the lining surfaces of the peritoneal (abdominal) cavity.
(Hyperthermic means that the solution, in which the chemotherapy is mixed, is heated to above normal body temperature : Intra-peritoneal means within the abdominal cavity).
Before HIPEC is administered, the surgeon will remove all visible tumors that can be removed from within the peritoneal cavity (cyto-reductive surgery). The surgeon will then continuously circulate a heated sterile solution–containing chemotherapy–throughout the peritoneal cavity, for a maximum of two hours. This procedure is designed to try to kill any remaining cancer cells. By delivering therapy directly into the area affected by cancer, HIPEC is thought to improve chemotherapy absorption in that area and reduce potential whole body (systemic) side-effects.
Chemotherapy may be considered following surgery or following cyto-reductive surgery & HIPEC – particularly where there may be a high risk of the cancer returning.
In inoperable, recurrent or metastatic disease, palliative (that is, non-curative) chemotherapy may be offered to try to gain disease control and /or provide symptom relief. The chemotherapy used would be similar to that used in colorectal cancer.
Clinical research and safe new treatment development is essential to provide best care for those with Neuroendocrine Cancer – we need to know that treatments not only work but work safely. There are several phases of trial therapy – further information can be found in our “Clinical Trials” factsheet. Each trial will have specific criteria in regards to patient suitability – this can be discussed with your clinical team. You do not have take part in a trial – participation is voluntary.
Managing symptoms, including pain, is an important part of total care – and therefore occurs throughout care, not just at ‘end-of-life’. Symptom control or ‘palliation’ refers to what is used to alleviate or reduce the impact your cancer, other health issues and /or treatments may be having on you and your physical and mental health. It can include anything from simple medication and / or a combination of some of the treatments mentioned above to counselling and practical support.
There are expert agreed guidelines regarding how and when follow up should occur, however, in practice this varies and often with good reason. Follow up should be expert informed & evidence /research based but also tailored to you and what is appropriate for your best care.
Consideration should be given to refer all patients diagnosed with GCC to a national peritoneal cancer service (HIPEC) for MDT review of diagnosis and treatment plan development.
The scheduled follow-up for patients after curative surgical treatment should include clinical, biochemical and imaging every 3 – 6 months, then yearly, as per guidelines for colorectal adenocarcinoma.
Gastrointestinal follow-up may also be recommended because of the reported association of GCC (up to 48%) with gastro- intestinal neoplasms.
Some authors recommend lifelong surveillance as significant risk of relapse remains after 5 years.
Referral to national peritoneal cancer services for MDT review or advice, regarding cyto-reductive surgery +/- hyperthermic intra-peritoneal chemotherapy (HIPEC) should be considered Patients who may benefit the most are incompletely resected GCC with stage II/III/IV disease.
Advanced disease: follow up as per guidelines – but should be guided by prognosis, expected treatment efficacy and treatment related toxicity. Your health, well-being, physical activity, informed choice and preference for ongoing care as well as aim of treatment should be reviewed and discussed to best plan care.