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Neuroendocrine cancer of the stomach is rare and can affect people in different ways, depending on the type of cancer.
Some types grow slowly and may cause few symptoms, while others can be more aggressive. Symptoms may include abdominal discomfort, changes in appetite or unexplained weight loss. Tests and scans can help your healthcare team understand the type of cancer and plan the most appropriate treatment and follow-up care.
This page explains more about neuroendocrine cancer of the stomach, including the different types, possible causes and symptoms, how it is diagnosed and monitored, and the treatments you may be offered. It also includes information about living with the condition and taking an active part in your care.
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
Treatment and outlook depend on factors including tumour type, grade, stage, differentiation and whether the cancer has spread.
This page looks at neuroendocrine cancer of the stomach.
Watch our video: Stomach Neuroendocrine Cancer
Neuroendocrine cancer of the stomach
Neuroendocrine cancer of the stomach is rare, comprising less than 2% of all stomach cancers. Among these cases, there are three primary types, with the possibility of a fourth:
- Type I: this is the most common type (70–80%), typically presenting as small lesions, often less than 10 mm in size and occuring in multiples. They tend to be non-aggressive, well-differentiated and rarely metastasise. Type I neuroendocrine cancers may be associated with atrophic and/or auto-immune gastritis and/or H. Pylori infection.
- Type II: accounting for approximately 7–10% of cases, type II neuroendocrine cancers share similarities with type I, including their small size and potential multiplicity. They are usually slow growing and well-differentiated, but have a slightly higher likelihood of metastasis. Type II neuroendocrine cancers may be associated with Zollinger-Ellison syndrome (ZES) and/or multiple endocrine neoplasia 1 (MEN1).
- Type III: representing roughly 20% of cases, type III neuroendocrine cancers typically manifest as solitary, larger lesions (often two or more centimetres in size). They can exhibit well- or poorly differentiated cells and tend to grow more rapidly than type I or II, making them more likely to metastasise.
Some experts suggest formally subdividing type III based on cancer cell differentiation, distinguishing between well- and poorly differentiated forms.
Symptoms
Symptoms can vary, depending on the grade, stage, and underlying causes.
Common symptoms:
- Stomach discomfort/pain
- Reflux: regurgitation, heartburn, dyspepsia
- The feeling of fullness in the upper abdomen after eating (early satiety)
- Reduced appetite
- Unintentional weight loss
- Anaemia (low iron and/or B12)
- Stomach and/or duodenal irritation leading to nausea and/or vomiting (vomit may or may not contain blood)
- Darkening of faeces (black or very dark stools).
Type-specific symptoms:
- Type I stomach neuroendocrine cancer: typically not associated with neuroendocrine cancer-associated syndromes.
- Type II stomach neuroendocrine cancer: may be associated with ZES and/or MEN1 symptoms.
- Type III stomach neuroendocrine cancer: carcinoid syndrome may develop if the disease spreads to the liver (metastasises), although this occurrence is rare.
Rare symptoms, including paraneoplastic syndrome and oncological emergencies, such as raised calcium levels (hypercalcaemia), may occur.
For further details about MEN1, you can find valuable information at the Association for Multiple Endocrine Neoplasia Disorders (AMEND).
Types of tests
Tests that may be used to diagnose and monitor neuroendocrine cancer of the stomach:
- Type I: this type is often associated with low vitamin B12 levels, the presence of anti-parietal cell antibodies, and anti-intrinsic factor antibodies. Gastric pH may be high (>4).
- Type II: this type is linked to ZES and gastrinomas, often occurring as part of MEN1. While vitamin B12 levels may be normal, iron deficiency is common. Anti-parietal cell antibodies may test negative, but gastrin levels (when not taking proton pump inhibitors) are typically high, although gastric pH may be low (<2).
- Type III: this type is not typically associated with elevated gastrin levels, despite the presence of ulceration.
Bloods and urine
- Full blood count
- Vitamin B12 (and serum iron)
- Parietal cell and intrinsic factor antibodies
- Liver and kidney function
Biochemical:
- Chromogranin A (and B)
- Urinary 5-HIAA
- Gastric pH
- Genetic analysis is recommended in case of suspected MEN1
Scans and other tests
- Upper endoscopy – oesophagogastroduodenoscopy (OGD) with or without biopsy
- Endoscopic ultrasound (EUS) with or without biopsy
- CT or MRI (for type III)
- Octreotide (SPECT) scan or Gallium-Dotatate PET/CT (type II and III)
- FDG-PET if high grade or rapidly progressing disease
Watch our video: Your Guide to Scans
Pathology (what can be seen through special tests under a microscope)
- Differentiation and cellular morphology
- Haematoxylin and eosin
- Synaptophysin
- Chromogranin
Expert recommendation for type I disease includes biopsying the background mucosa to check for evidence of dysplasia.
Diagnosis and grading
Stomach neuroendocrine cancer is a complex condition with various subtypes and behaviours. Each diagnosis is unique, and understanding your situation is essential for making informed decisions about your care. It’s important to remember that not all cancers are the same – some may grow slowly, while others are more aggressive. This diversity underscores the significance of understanding your diagnosis and its implications.
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.
Understanding grading
Cancers vary significantly in their characteristics and behaviour. Differences in growth speed, invasiveness into nearby tissues, and the potential for spreading to other parts of the body exist among different cancer types. While some cancers progress slowly and may not have invaded nearby structures or metastasised, they still harbour the potential to do so. Conversely, certain cancers may have already spread by the time of diagnosis.
Cancer grading indicates the rate of its growth. You might come across terms like ‘Ki67’ or ‘Mitotic Index’ in your clinic letters or medical reports, which are indicators of the cancer’s grade. This classification spans from Grade 1, indicating slow growth, to Grade 3, signifying a more aggressive and rapidly progressing disease. Understanding the grade offers valuable insights into the behaviour and potential course of the neuroendocrine cancer.
How is neuroendocrine cancer of the stomach treated?
When it comes to planning treatments for all patients, several factors come into play. Your treatment plan will be tailored specifically to you, considering the type of neuroendocrine cancer of the stomach you have. Even if your diagnosis seems similar to another patient’s, your treatment and follow-up plan may differ.
Your healthcare team will engage in thorough discussions about your treatment options, providing you with both written and verbal information to ensure you can make an informed decision. Together, you and your medical team can determine the most suitable treatment for your unique circumstances. It’s widely agreed that all neuroendocrine cancer patients should undergo review by a specialist multidisciplinary team (MDT).
The choice of treatment options depends on various factors, including the type, location, and size of your neuroendocrine cancer, as well as whether it has spread and to what extent. Additionally, your overall health, any other medical conditions, and your general fitness will be considered. When meeting with your doctors or specialist nurse/team, the primary goal is to provide you with the information necessary to understand your condition fully.
The primary goal of treatment is to provide you with the best possible care and quality of life. This involves access to appropriate treatments, symptom management, and addressing your unique priorities.
The choice of treatment options depends on several factors, including the type, location, and size of your cancer, as well as whether it has metastasised. Additionally, your overall health and any other medical conditions will be considered.
Your treatment plan may include one or more of the following approaches:
- Surgery
- Disease control to slow or halt further growth and spread
- Palliative care to alleviate any symptoms you may be experiencing
Surveillance
Monitoring through regular clinic reviews, blood tests and scans serves as a valuable means to assess the effectiveness of your treatment or during intervals between treatments, which may extend for months or even years. Since not everyone requires ongoing treatment, surveillance plays a crucial role in periodically evaluating your cancer and overall health for any signs of change that might warrant consideration of treatment.
It’s worth noting that all treatments carry potential side effects. Therefore, it’s essential to determine when treatment would be beneficial for you and when it might not be necessary.
Surgery
The approach to treating your neuroendocrine cancer depends on factors such as its location, size, position and extent of spread. Stomach cancers can manifest in different ways – some develop within polyps, while others form solid tumors.
Polyps, which are small cell clumps, can take two forms:
- Pedunculated polyps: these polyps hang from a short stalk.
- Sessile polyps: these are flat and grow directly from the surrounding tissue.
If your neuroendocrine cancer is within a polyp, it may be possible to remove it through:
- Polypectomy: this involves the removal of polyps during endoscopy using hot or cold forceps or a snare.
- Endoscopic mucosal resection (EMR): during EMR, an endoscope is inserted at the site of the polyp. A small needle is used to inject fluid beneath the polyp, creating a safe area for its removal using a snare. The site is then cauterised to prevent bleeding.
Endoscopic submucosal dissection (ESD) is similar to EMR but employs a small knife instead of a snare to remove the polyp, simultaneously cauterising blood vessels to prevent bleeding.
If endoscopic removal is not possible for your neuroendocrine cancer, open surgery may be necessary, which may involve:
- Sub-total gastrectomy: this procedure removes a portion of the stomach through an abdominal incision.
- Total gastrectomy: in this case, the entire stomach is removed through a similar abdominal incision.
Non-surgical treatment options
Somatostatin analogs (SSAs)
SSAs can play a crucial role in regulating hormone secretion when abnormal levels are being produced. Additionally, SSAs may be employed to decelerate the growth rate in cases of low to moderate-grade ‘well-differentiated’ neuroendocrine cancer.
Chemotherapy
Chemotherapy can be administered either orally (in tablet form) or intravenously (through a vein) with the aim of slowing tumour growth or reducing tumour size. In high-grade disease, particularly in ‘poorly differentiated’ neuroendocrine carcinomas (NECs), it may be the first-line therapy or used in combination with other treatments. Additionally, chemotherapy may be employed to increase tumor cell sensitivity to radiation therapies.
Targeted molecular therapies
Chemotherapy can be administered orally (in tablet form) or intravenously (through a vein) to slow tumour growth or attempt to reduce tumor size.
Peptide receptor radionuclide therapy (PRRT)
Sometimes also called radioligand therapy or targeted radiation therapy, PRRT utilises focused radiation to target and treat neuroendocrine cancer cells. It is often considered for patients who have received a ‘positive’ Octreotide or Gallium scan, indicating ‘receptor-positive’ disease.
Endoscopic procedure
Endoscopic treatment, such as the removal of a stomach or rectal polyp, can be performed via endoscopy.
Interventional radiology
Techniques like embolisation or ablation can be employed to treat neuroendocrine cancer that may develop in the liver and/or lungs.
Clinical trials
Clinical research and the development of safe, new treatments are essential to provide the best care for those with neuroendocrine cancer. It’s crucial to ensure that treatments not only work but are also safe.
Clinical trials involve several phases. Each trial has specific criteria for patient suitability, which you can discuss with your clinical team.
Participation in a trial is entirely voluntary, and you are not obligated to take part.
Symptom control
Managing symptoms, including pain and underlying conditions, is an integral component of comprehensive care. This approach is applied throughout the entire care pathway.
Symptom control, often referred to as ‘palliation’, aims to alleviate or reduce the impact of your cancer, other health concerns, and treatments on your physical and mental wellbeing. Palliation encompasses a range of options, from simple medications to a combination of the treatments mentioned above, as well as counseling and practical support.
Follow-up and management
While there are expert-agreed guidelines regarding how and when follow-up should occur, it’s important to note that practical approaches can vary, often with good reason.
Follow-up care should be expert informed, evidence/research based, and tailored to your unique needs for the best possible care.
For patients with neuroendocrine cancer of the stomach, especially following excision:
- Type I: conservative management typically involves lab and clinical control every 6–12 months, as clinically indicated. Additionally, it may include OGD with biopsies and/or polyp resection every 12–24 months.
- Type II: endoscopy should be repeated yearly along with lab monitoring. Polyps less than 1 cm can be managed similarly to type I.
- Type III and IV: patients who have undergone sub-total or total gastrectomy should follow the programmes indicated for gastric adenocarcinomas.
- Type III and IV (inoperable or metastatic): patients in this category should follow the adenocarcinoma protocol, taking into account general health, symptoms, treatment options and individual preferences.
In cases of advanced disease, follow-up should align with guidelines, but it should also be guided by prognosis, expected treatment efficacy, and treatment-related considerations.
Your overall health, wellbeing, physical activity, informed choices and preferences for ongoing care should be reviewed and discussed to plan your care optimally.
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.
Causes and risk factors
While the exact causes of neuroendocrine cancers remain unknown, ongoing research aims to gain a comprehensive understanding of both the pathology and biology, offering hope for improved insights into their origins.
Although the majority of neuroendocrine cancers are not hereditary, certain rare conditions can elevate your risk. If other family members have been diagnosed with a specific cancer, particularly at a young age (50 years or younger), or if there is a history of cancer-associated genetic conditions in your family, it’s essential to inform your specialist team. This ensures that they have a complete picture of your medical history, including any family medical illnesses or conditions.
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 stomach 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.
