Supporting the

Neuroendocrine Cancer Community

Neuroendocrine of the Pancreas

Here, you’ll find a variety of resources tailored to those affected by neuroendocrine cancer of the pancreas. We offer information on causes, symptoms, diagnosis, and treatment options. Dive into inspiring patient stories, stay updated on research developments, and access expert insights. Our community is here to support you, providing both emotional guidance and practical advice.

Explore treatment approaches, connect with others who understand your journey, and discover ways to lead a fulfilling life during and after treatment. You’re not alone in this – together, we’re here to educate and support.

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The Facts

Neuroendocrine cancer is a type of cancer that originates in the specialised cells of the neuroendocrine system, which can be found throughout the body and produce hormones that regulate various bodily functions.

Neuroendocrine Neoplasm (NENs) serves as an umbrella term encompassing a cluster of cancers originating in neuroendocrine cells. These cancers might also be denoted as NETs, NECs, or Carcinoids. NENs manifest when neuroendocrine cells deviate from their normal function, undergoing abnormal growth or behaviour.

Two principal types of NEN exist:

1. NETs (neuroendocrine tumours): Characterised as ‘well-differentiated‘, these tumours usually exhibit a gradual to moderate growth pattern.

2. NECs (neuroendocrine carcinomas): Identified as ‘poorly differentiated‘, these tumours tend to grow rapidly.

To simplify matters, we employ the term NENs throughout this page.

Select play to watch this short video about Pancreatic Neuroendocrine tumours by Professor Martyn Caplin.

The Pancreas

The pancreas is a large gland, that is located behind the stomach and joins the digestive tract via the main pancreatic duct. It has a number of different types of cells that are responsible for producing substances (enzymes, peptides, hormones) that play an essential role in converting the food we eat into fuel for the body’s cells, as well as regulating our blood sugars.


Pancreatic Neuroendocrine Cancer Symptoms:

Neuroendocrine Cancer may or may not present associated syndromes. In cases of Pancreatic Neuroendocrine Cancer, you might hear the terms “Functioning” (with hormone-related symptoms) or “Non-functioning” (without hormone-related symptoms). These terms can also refer to whether the cancers appear on nuclear medicine imaging (see diagnostic tests).

Non-Functioning Pancreatic Neuroendocrine Cancers:
Most of these cancers are non-functioning, meaning they do not overproduce hormones. Symptoms, if they occur, are often related to the cancer’s size and location, such as back pain, jaundice, stomach pain, and weight loss.

Functioning Pancreatic Neuroendocrine Cancers:
These tumours overproduce specific gut hormones, leading to syndromes:

  1. Insulinoma (Insulin): Symptoms include dizziness, sweating, hunger, confusion, and irritability. Symptoms may improve with eating, potentially leading to weight gain.
  2. Gastrinoma (Gastrin): Known as Zollinger-Ellison syndrome, it causes acid reflux, heartburn, stomach/chest pain, diarrhoea, and low haemoglobin, causing tiredness.
    3. VIPoma (Vasoactive Intestinal Polypeptide): Results in very watery, frequent, and high-volume diarrhoea, along with changes in blood potassium levels and low stomach acid.
    4. Glucagonoma (Glucagon): Causes a skin rash known as NMES (Necrotising Migratory Erythema Syndrome), symptoms like diabetes, diarrhoea, blood clots, and changes in skin, nails, and hair.
    5. Somatostatinoma (Somatostatin): Can mimic diabetes symptoms, along with diarrhoea, steatorrhea (fatty pale stools), and weight loss.
    6. PPoma (Pancreatic Polypeptide): Rarely produces a syndrome.

Other Neuroendocrine Cancer Associated Syndromes: These result from the unusual production of non-pancreatic hormones (ectopic hormone production):

  • Cushing’s Syndrome (ACTHoma)
  • Hyperparathyroidism (PTHrPoma)
  • Calcitoninoma (watery diarrhoea and facial flushing)
  • Neurotensinomas (low blood pressure, flushing, diarrhoea, weight loss, and diabetes)
  • GRFoma (associated with acromegaly)

Additional Symptoms and Considerations:
In addition to the above syndromes, other rarer symptoms, including Paraneoplastic syndrome and oncological emergencies (such as Hypercalcemia), may occur.




Tests that may be used for the diagnosis and/or monitoring of Neuroendocrine Cancer of the Pancreas:

Blood and/or urine:

  • Full blood count
  • Liver and kidney function
  • B12/Iron/Ferritin/Folate
  • Chromogranin A
  • Urinary or serum 5HiAA (serotonin).
  • Gut Hormone Profile
  • Calcium
  • Calcitonin.

Scans and other tests:

  • CT chest, abdomen and pelvis and/ or CT chest & MRI abdomen and pelvis
    Octreotide (SPECT) or Gallium-Dotatate PET/CT.
  • If available, a GLP 1 scan may be beneficial in Insulinoma
  • Endoscopic Ultrasound (EUS).

Pathology (what can be seen through special tests under a microscope):


  • Differentiation and cellular morphology
  • Synaptophysin
  • Chromogranin
  • Ki67
    With or without specific hormone staining.

Diagnosis and Grading

Receiving a diagnosis of pancreatic neuroendocrine cancer can be an unexpected and challenging moment in your life. We understand the range of emotions and questions that may arise during this time, and we’re here to provide you with the support and information you need.

Pancreatic 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.

Our ‘Just Diagnosed’ page is designed to be your go-to resource during this pivotal phase. We offer valuable information on what to expect, access to specialist care, and emotional support.

More than just information, our website also connects you with a supportive community. Sharing experiences with others who have faced similar challenges can be profoundly comforting and enlightening. We encourage you to explore our online community groups and support groups, where you can find understanding, advice, and a network of individuals ready to uplift you.

Remember, you’re not alone on this pathway. Together, we can navigate the intricacies of pancreatic neuroendocrine cancer and work towards the best possible outcomes. Visit our ‘Just Diagnosed’ page to access vital resources and connect with a community that cares. Your well-being is our priority, and we’re here to stand by your side every step of the way.

Click “Play” to view Clinical Nurse Specialist, Mike Tadman’s insightful presentation from the 2020 NCUK Virtual Summit: “Spotlight on Pancreatic Neuroendocrine Cancers – 2020.”

Understanding Grading

Not all cancers share the same characteristics or exhibit uniform behaviour. Among them, there exist variations in growth speed, invasiveness of nearby structures, and propensity for spreading to other body parts upon diagnosis. While some cancers grow gradually and may not have infiltrated surrounding structures or disseminated, they still possess the potential to do so. On the other hand, certain cancers may have already spread by the time of diagnosis.

The grade of cancer indicates the rate of its growth. You might encounter terms such as ‘Ki67’ or ‘Mitotic Index’ in your clinic letters or medical reports indicating the grade. This classification ranges from Grade 1, representing a slow-growing nature, to Grade 3, signifying a more aggressive and rapidly progressing disease. Understanding the grade provides crucial insights into the behaviour and potential trajectory of the NEN.

How is Panreactic Neuroendocrine Cancer Treated?

Personalised treatment planning is essential for all patients and is tailored to your specific type of NEN. Even if your diagnosis sounds similar to another patient’s, your treatment and follow-up plan may differ significantly.

Your dedicated care team will discuss treatment options with you, providing written and verbal information to help you make informed decisions. Together, you can determine the most suitable treatment. It’s important to note that a consensus exists that all NEN patients should undergo review by a specialist Multidisciplinary Team (MDT).

Treatment choices depend on factors such as NEN type, location, size, spread, overall health, and any other medical conditions. When meeting with your doctors or specialist nurse/team, their goal is to ensure you have the information necessary to understand your condition fully.

Treatment Options

The primary goal of treatment is to provide you with the best possible care and quality of life. This includes access to appropriate treatments, symptom management, and addressing your individual priorities. Treatment decisions are tailored to your specific NEN type.

Personalised Treatment:
Even if your diagnosis sounds similar to another patient’s, your treatment plan may differ significantly. Your care team will discuss treatment options, ensuring you receive written and verbal information to make informed choices.

Factors Influencing Treatment:
Treatment options depend on various factors:

  • NEN type
  • Tumour location and size
  • Spread of the disease
  • Other health concerns or illnesses
  • Your overall health and fitness.

Treatment Approaches:
One or more of the following approaches may be suggested:

1. Surgery:

  • Enucleation
  • Distal pancreatectomy
  • Whipples procedure (Pancreatoduodenectomy)
  • PPPD procedure (similar to Whipples but excludes pyloric sphincter removal)
  • Total Pancreatectomy
  • Multi-visceral Pancreatic Resection (for NETs spreading to other organs).

SELECT PLAY. In this insightful session, Pancreatic Surgeon, Zahir Soonawalla, shared valuable insights during our Natter Support Group meeting. His presentation focused on pancreatic neuroendocrine tumours (NETs) and the surgical approaches for managing them. The session was recorded on 21.02.23.

2. Non-Surgical Treatments:

  • Somatostatin analogues (SSAs)
  • Chemotherapy
  • Targeted Molecular Therapies
  • Peptide receptor radionuclide therapy (PRRT)
  • Radiotherapy
  • Endoscopic procedures
  • Interventional radiology
  • Irreversible electroporation (IRE or Nanoknife)
  • Clinical Trials.

3. Symptom Control (Palliation):
Symptom management, including pain relief, is a crucial aspect of care throughout your journey. Palliation aims to alleviate the impact of your cancer and treatments on your physical and mental well-being.

Clinical Trials:
Participating in clinical trials can contribute to the development of safe and effective treatments. Clinical research is essential to provide the best care for NEN patients.

Pancreatic Exocrine Insufficiency (PEI) & Pancreatic Enzyme Replacement Therapy (PERT)

  • Pancreatic Exocrine Insufficiency (PEI) can lead to malnutrition due to nutrient malabsorption, underlying pancreatic disease effects, and symptom impact on oral intake. Symptoms include steatorrhea (pale, fatty/greasy, loose stools), weight loss, diarrhoea, abdominal pain, and bloating.
  • Pancreatic Enzyme Replacement Therapy (PERT) is the primary treatment and significantly improves survival and quality of life (QoL) in PEI patients.
  • Pancreatic enzyme replacement therapy (PERT) is the cornerstone of treatment and is associated with improved survival and quality of life (QoL) in patients with PEI.

Causes and / or risk factors

We do not know exactly what causes NENs, however research is ongoing, to truly understand both the pathology and the biology with the hope that this will lead to a much better understanding of the causes.

Most NENs do not run in families; however, there are a number of rare conditions that may increase your risk. We recommend speaking to your GP, and specialist team, if you have a family history of:

  • MEN1 (Multiple Endocrine Neoplasia 1 – also known as Wermers Syndrome)
  • VHL (Von-Hippel Landau)
  • NF1 (Neurofibromatosis 1)
  • and / or Tuberous Sclerosis.

They may refer you for an NHS genetic test, which will tell you if you have inherited one of the cancer-risk genes – if this has not already been done. Up to 30% of Pancreatic NENs occur on the background of one of these particular genetic conditions.

Follow up

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.

Follow up is dependent on grading and completeness of resection (if undertaken):

  • G1: complete resection (R0) / no lymph nodes : post operative check and evaluation.
  • G1: incomplete resection (R1/node positive) & G2 : see at 3, 6 and 12 months then annually if stable : labs & CT/MRI.
  • EUS +/- Functional imaging (at 1yr then 3 yearly unless recurrence/ progression is suspected – optional).
  • G3: review 2 – 4 monthly if on ongoing (or may fit criteria) for treatment. FDG-PET as preferred mode of functional imaging in high grade disease.
  • If inherited disorder present/suspected: follow up as per disorder e.g. MEN1, VHL, TS, NF1.


  • G1/G2: 3 – 9 monthly intervals in first 2 years – then at intervals dependent on presence / absence of disease / functionality (see below) – min annual CT + bloods
    (EUS/functional 3 yearly unless recurrence suspected)
  • G3: 2-4 monthly on treatment / potential for treatment

By type:

  • Gastrinoma: Routine + B12, ionised calcium, PTH, Gastrin – with annual assessment for Cushing syndrome
  • Insulinoma: Routine + Fasting glucose, Insulin, C-Peptide, Pro-Insulin, β-hydroxybutyrate
  • Others e.g. VIPoma, PTHRPoma, etc.: Routine + Gut Hormone Profile.
  • If no metastatic disease : 3-6 monthly then annual follow up with specific biomarkers, CT/MRI
  • (EUS/functional 3 yearly unless recurrence suspected).

If metastatic disease present:

  • 3-6 monthly on treatment/ potential for treatment : with specific biomarkers, CT/MRI .
  • (EUS +/- re-biopsy and / or functional imaging in progressive disease).
  • If inherited disorder present/suspected: follow up as per disorder e.g. MEN1, VHL, TS, NF1.

Advanced disease – functioning or non-functioning:

  • 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.