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Neuroendocrine cancers of the appendix are rare cancers that develop from neuroendocrine cells in the appendix, a small pouch where the small and large bowel meet. They include neuroendocrine tumours (NETs) and the much rarer neuroendocrine carcinomas (NECs).
Neuroendocrine cancers of the appendix may not cause specific symptoms and are often discovered after surgery for suspected appendicitis. Tests and scans can help your healthcare team understand your individual cancer and plan the most appropriate treatment and follow-up care.
This page explains more about neuroendocrine cancers of the appendix, including possible causes and symptoms, how they are diagnosed and monitored, and the treatments you may be offered. It also includes information about living with your diagnosis and where you can find further information and support.
Use the links in the box to find the information that is most relevant to you.
What is neuroendocrine cancer?
Neuroendocrine cancer is a type of cancer that begins in the neuroendocrine cells, which are found throughout the body. These cells have the ability to release hormones that can regulate many body functions. When they become cancerous, their growth rate and normal function is disrupted.
There are two main types:
- Neuroendocrine tumours (NETs) – usually slower growing
- Neuroendocrine carcinomas (NECs) – less common and faster growing
This page looks at both NETs and NECs of the appendix.
Goblet cell cancer of the appendix
Goblet cell cancer (GCC) of the appendix is no longer classed as a neuroendocrine cancer.
Even though GCC of the appendix contains a mix of different cell types, including some neuroendocrine cells, experts now recognise that it behaves much more like a typical bowel-type cancer called an adenocarcinoma (a cancer of gland-forming or mucus-producing cells).
Because of this, research and international guidelines have officially reclassified it as a type of adenocarcinoma, and it is now staged, monitored, and treated in the same way as other adenocarcinomas.
Further information on GCC of the appendix can be found at:
Your appendix
The appendix is a small, finger-shaped pouch that sits on the lower right side of the abdomen, where the small and large bowel meet. It is usually about 9 cm (4 inches) long.
Although it was once thought to have no real purpose, we now know the appendix can play a helpful role in the body. It acts as a safe ‘reservoir’ for healthy gut bacteria and supports the immune system by helping the body respond to infections in the digestive tract.
Because the appendix is narrow, it can sometimes become blocked, leading to inflammation called appendicitis, which may require surgical removal.
The good news is that people can live completely normal, healthy lives without an appendix, as the body adapts and other systems take over its functions.
Rarely, cancer may develop in the appendix. Neuroendocrine cancer is one of these rare cancers of the appendix and usually falls into two main types:
- Neuroendocrine tumours (NETs), which are the more common and usually slower growing
- Neuroendocrine carcinomas (NECs), which are much rarer, but can be more aggressive
How do neuroendocrine cancers of the appendix develop?
For most people, there is no clear cause and there are no known inherited or genetic risk factors.
Researchers are continuing to learn more about how the body works, how our environment may affect our health, and how genetic changes can lead to cancer.
This growing knowledge is helping to improve diagnosis and treatment and may lead to better ways to prevent cancer in the future.
Signs and symptoms
Many people with a neuroendocrine cancer of the appendix do not have specific symptoms linked to the tumour itself: if symptoms occur, they are usually similar to appendicitis.
1. Symptoms caused by the tumour itself (e.g. appendicitis)
Symptoms may begin with a pain in the middle of the abdomen that comes and goes, before moving to the lower right‑hand side where the appendix is located, where it can become constant and more severe. The pain may worsen with movement, coughing or pressing on the area. Some people may also lose their appetite, feel sick, or occasionally experience diarrhoea.
2. Symptoms caused by hormone changes
Neuroendocrine tumours (NETs) of the appendix very rarely cause changes in hormone levels. In fewer than 1 in 100 people (less than 1%), the tumour may produce extra hormones that lead to a group of symptoms called carcinoid syndrome. This is especially uncommon in the early stages.
Less than 1% (1 in 100 people) may experience carcinoid syndrome:
- Dry flushing (primarily on the face/neck – but can spread body wide)
- Diarrhoea
- Wheezing
- Fast heartbeat
- Other symptoms that may also occur – but rarely – include night sweats and/or allergy-like symptoms, such as itchy/irritated skin, watery eyes/runny nose (rhinitis), and/or swelling of the salivary glands
Carcinoid crisis (rare) may occur in those who have carcinoid syndrome
Carcinoid crisis can happen during anaesthesia or tumour manipulation (biopsy, surgery or interventional radiology – e.g. embolisation or ablation treatments) or be triggered by mental/physical stress.
Symptoms may be similar to carcinoid syndrome but are much more severe. These include severe flushing often accompanied by palpitations or a fast, irregular heart rate, low blood pressure, confusion and breathing difficulty, and can be life-threatening.
Specialist teams plan care to reduce this risk.
If you are at risk of carcinoid crisis, carry a Carcinoid Crisis Card so healthcare professionals know how to manage your care in an emergency. You can order one from us for free.
3. Rare symptoms (paraneoplastic syndromes)
Very rarely, cancer can trigger symptoms caused by the body’s immune response to cancer. These are uncommon but can sometimes cause serious health problems.
Watch our video: Paraneoplastic Syndromes – Explained
4. Symptoms related to treatment
Treatments can cause side effects, but not everyone experiences them.
Your team will talk through possible side effects and how they can be managed.
Tests and scans
Blood and urine tests
- Routine bloods, such as full blood count, liver and kidney function, and others to check overall health
- Chromogranin A (CgA) with or without chromogranin B (CgB)
- 5-HIAA or serotonin (NET only)
- NT-ProBNP may be taken if you have a history of heart disease or carcinoid syndrome (NET only)
- Vitamin, mineral and trace element check
Scans and procedures
A neuroendocrine tumour of the appendix is most often found after surgery for suspected appendicitis. However, some people may have had surgery for another reason and, as part of their checks before surgery, may have had tests such as an endoscopy or a computed tomography (CT) scan.
An endoscopy (sometimes called a ‘scope’) uses a long, thin, flexible tube with a light and camera on the end. This allows doctors to look inside the body on a screen and check for any changes or problems. Scopes can also be used to take small samples (called biopsies) or give treatment if needed.
Different types of scopes look at different parts of the bowel:
- A colonoscopy looks inside the large bowel (colon)
- A sigmoidoscopy looks at the lower part of the large bowel
Scans you may have:
- Chest X-ray, ultrasound scan
- CT, MRI scans
- Gallium-Dotatate CT PET scan (NET only)
- FDG-PET scan (NEC only)
- Echocardiogram (if you have a history of heart disease or evidence of carcinoid syndrome)
Watch our video: Your Guide to Scans
Histopathology
This means looking at cells and tissues under a microscope to understand disease. It helps confirm whether cancer is present, where it started, what type of cells are involved, and how quickly the cells are growing. These tests help confirm diagnosis and guide treatment.
Cancer gene changes and targeted treatments
Some neuroendocrine cancers (particularly NECs) may have specific cancer-cell gene changes (mutations or fusions) that can be targeted with treatment. For example PD-L1 or DLL-3.
Doctors may suggest a detailed test called genomic testing to look for these changes. Finding them may help match you with targeted treatments that are more personalised.
Treatment options
Your care is individualised based on your tumour, health and preferences.
1. Monitoring (surveillance)
Often used after treatment or if treatment is not possible but intervention or supportive care may help with symptom management. Regular scans and check-ups help monitor for any changes.
2. Surgery
For many people, having the appendix removed (an appendicectomy) is the only treatment that is needed for a NET of the appendix. In these cases, no further treatment may be required apart from a routine check-up after surgery. This is because most appendix NETs are small and will have been completely removed by appendicectomy.
However, sometimes further treatment may be considered, depending on the results from the laboratory (histopathology) after surgery:
- If the diagnosis is a NET: further surgery (to remove part of the bowel and nearby lymph nodes) may be recommended if the tumour is larger than 2 cm or if there are concerns that it may have spread or was not fully removed.
- If the diagnosis is a NEC: this is a rarer and more aggressive type of cancer. If there are concerns about spread or incomplete removal, chemotherapy may be discussed instead of further surgery as part of treatment.
Note that most appendix NETs are cured with surgery alone. Further treatment depends on tumour size, type and whether it has spread. Your healthcare team will explain what is best for your individual situation.
3. Non-surgical treatment options
Non-surgical treatments will be different depending on whether the diagnosis is NET or NEC.
NET treatments
If NET was incompletely removed or has spread and further surgery is not possible:
- Somatostatin analogues (SSAs): to control hormone-related symptoms and/or tumour growth.
- Targeted medical therapy (e.g. the mTOR inhibitor everolimus): these are targeted treatments that block a protein that cancer cells use to grow and divide, helping to slow or stop tumour growth.
- Chemotherapy: rarely used as a first treatment for low grade (G1-2) NETs but may be considered in G2-3 or if other treatments are not suitable or have stopped working. It may be given as tablets or through a drip into a vein. For example CapTem (capecitabine + temozolomide) tablets or, rarely, Streptozocin/5FU via a drip.
- Radiotherapy: external beam radiation therapy (EBRT) may be used to treat symptomatic bone metastases.
- Radioligand therapy (RLT, also known as peptide receptor radionuclide therapy (PRRT) or lutetium‑177): mainly used for metastatic or progressing disease. It is a targeted internal radiation treatment. It delivers radiation directly to cancer cells, usually given in four sessions spaced 8–12 weeks apart.
- Symptom control and supportive care: includes any treatment or support that helps you feel better, even if it is not directly treating the cancer. This may include medicines, dietary advice and/or psychological support. Techniques such as mindfulness or relaxation may help with pain, fatigue, stress or anxiety.
- Clinical trial therapy: clinical trials are used to find out if new treatments and techniques are safer and more effective than the ones that already exist, or for discovering if existing treatments can be used in different, more effective, ways or combinations.
NEC treatments
These may be considered as first line treatment if diagnosed before surgery or following surgery if NEC was not suspected and has been incompletely removed, has spread or there is concern about recurrence.
- Chemotherapy: considered to be a first line treatment for NECs in inoperable disease. It may also be considered if surgery is not possible or after surgery – if it was incomplete or doctors think there is a high risk of recurrence. The main treatment is usually platinum-based chemotherapy, sometimes combined with etoposide (or irinotecan in certain cases). If the cancer comes back or grows, further treatment options may include reusing the first treatment or trying other chemotherapy combinations such as FOLFOX.
- Radiotherapy: external beam radiation therapy (EBRT) may be used to treat symptomatic bone metastases.
- Immunotherapy: clinical trials are looking promising in treating NECs, but it has yet to become a standard of care. Research is ongoing. Immunotherapy may be considered in NEC if available and accessible. They include: immune checkpoint inhibitors, which help your immune system recognise and attack cancer by removing the ‘brakes’ that normally hold it back, and DLL3 inhibitors, which target the protein DLL3, frequently over-expressed in high-grade neuroendocrine cancers.
- Symptom control and supportive care: includes any treatment or support that helps you feel better, even if it is not directly treating the cancer. This may include medicines, dietary advice and/or psychological support. Techniques such as mindfulness or relaxation may help with pain, fatigue, stress or anxiety.
- Clinical trial therapy: clinical trials are used to find out if new treatments and techniques are safer and more effective than the ones that already exist, or for discovering if existing treatments can be used in different, more effective, ways or combinations.
Diagnosis and living with neuroendocrine cancer of the appendix
A diagnosis of neuroendocrine cancer can bring questions, uncertainty and a range of emotions. Information and support are available to help you understand your diagnosis and navigate what happens next.
Just diagnosed with neuroendocrine cancer? Access information and support to help you navigate the early days after diagnosis.
Follow-up care
Follow-up is an important part of your ongoing care. It helps your healthcare team check how you are doing, spot any changes early, and support your physical and emotional wellbeing.
While expert guidelines offer a general plan for follow-up, your care will always be tailored to you and your specific type of neuroendocrine cancer.
At each appointment, your team will ask how you are feeling, review your symptoms and medications, and arrange blood tests if needed.
You may also be offered a holistic needs assessment (HNA). This is an opportunity to talk with your specialist nurse or doctor about any physical, emotional, practical, or social needs, so the right support can be put in place.
Guideline follow-up recommendations for appendiceal NET
If the NET:
- Was less than 1 cm in size
- Has been fully removed
- Shows no spread to lymph nodes or other concerning features
- Is low grade (slow growing)
Expert guidelines recommend no follow up beyond a post-surgery check-up. Discharge will be discussed with you and decided together with your healthcare team.
If the NET:
- Is between 1 and 2 cm in size
- Has been fully removed (including any further recommended surgery)
- Shows no spread to lymph nodes or other concerning features
- Is low grade (slow growing)
You may not need any further treatment or long-term follow-up. Discharge or short-term follow-up will be discussed with you and decided together with your healthcare team.
However, closer follow-up is recommended if the NET:
- Is larger than 2 cm, or
- Has not been fully removed, or
- Shows features that increase the risk of it coming back, or
- Has come back (recurred) or spread to other parts of the body
In these cases, you will usually have regular follow-up appointments:
- Every 3 to 12 months for the first 3 years
- Then every 1 to 2 years, for at least 8 years after diagnosis
Follow-up may include scans such as:
- Ultrasound or MRI (often preferred in younger people)
- CT scans or MRI (more commonly used in older people)
- Specialised scans (nuclear medicine imaging) if there is concern that the tumour has returned or is growing
Follow-up recommendations for appendiceal NEC
Follow-up usually comprises more intensive surveillance than for NETs.
Follow-up usually happens every 3 to 6 months for the first year, and then every 6 to 12 months in the long term. For example:
Early-stage (stages 0–1) NEC:
- Every 3 months during the first year
- Every 6 months during years 2 and 3
- Then once a year
More advanced disease (stages 2–4):
- Every 2 to 3 months during the first year
- Every 3 months during years 2 and 3
- Every 6 months during years 4 and 5
- Then once a year
But this should, as mentioned, be personalised to each individual’s health, wishes and preferences.
How you can be part your care
You are a key part of your team. You can help by:
- Reporting new symptoms or any changes in current symptoms or overall health
- Asking questions if at all uncertain or you want more information about your diagnosis and/or plan of care
- Attending appointments made – and checking up with the team if you haven’t received an appointment according to your agreed plan of care.
- Let your team know of your preferences and wishes regarding care – what is most important to you.
Support and further information
We have worked with the International Neuroendocrine Cancer Alliance (INCA) to provide information resources in more than 10 different languages: these are available here.
Stories from our community
Hear from people connected to neuroendocrine cancer as they share their experiences, perspectives and insights – from living with the condition to caring for, treating and supporting those affected.
Remember
Remember: every person’s experience with neuroendocrine cancers of the appendix is different – there may be shared similarities, but each journey is unique.
Your healthcare team will support you every step of the way, guiding your treatment, care, and follow-up. We’re here alongside them, offering additional support and information whenever you need it.
The information on this page is based on available clinical expert guidelines and publications. It is for informational purposes only and should not be considered professional medical advice.
