Recently diagnosed with
a gastroenteropancreatic
neuroendocrine tumor (GEP-NET)

Receiving a new cancer diagnosis

People can react in different ways to a cancer diagnosis.¹ If you or a loved one feels as if you are struggling physically or emotionally try to reach out to family, friends, or a support group.²

You may want to consider getting professional emotional support from a psycho-oncologist (a psychologist that specializes in addressing the needs of patients with cancer).^2,3 The availability of psycho-oncologists may differ from country to country; your clinical team or local patient organization may be able to provide more guidance around accessing this support.

If you have any questions, reach out to your doctor or clinical team, and use available support tailored to your needs when you require it.

The support you may receive consists of three pillars:

Medical support: focuses on managing the disease and controlling symptoms⁴

Psychological support: helps cope with symptoms, emotions and personal relationships³

Patient organizations: provide information, support, and the opportunity to share experiences with other NET patients, which can help with feelings of isolation^2,5

The organization below can help you find your nearest support groups and societies:

International Neuroendocrine Cancer Alliance (INCA)⁵ Find NET support groups for patients and carers all over the world

What are NETs?

NET stands for neuroendocrine tumor, a rare type of harmful growth made up of neuroendocrine cells you may not have heard of. NETs originate in the diffuse endocrine system and these cells are present throughout the body. As such, NETs can develop anywhere in the body, but most occur in the digestive tract and the lungs.⁶ The focus here will be on NETs in the digestive tract.

Healthy neuroendocrine cells produce hormones and other substances that help to keep the body functioning properly.⁷ But some NETs produce too many hormones, which can lead to a variety of symptoms - these are called functioning NETs.⁸

Most NETs don’t produce any substances that cause symptoms, and these are called non-functioning NETs.⁸ People with non-functioning NETs usually remain symptom-free until the tumor has spread to other parts of the body, or the size of the tumor starts causing problems in organs or tissue.^8,9 Functional NETs may also be asymptomatic in the early stages of disease.⁹

What are GEP-NETs?

GEP-NET stands for gastroenteropancreatic neuroendocrine tumor, a type of NET that’s specific to the gastrointestinal system. This includes the food pipe (esophagus), stomach, liver, pancreas, intestines, appendix and rectum.^8,10,11

You may have heard the terms foregut, midgut or hindgut. These are general terms for the area of the body in which the GEP-NET has originated from:^8,10

Foregut

Esophagus, stomach, liver, pancreas, and start of the small intestine (duodenum)

Midgut

Small intestine/bowel (jejunum and ileum), the first part of the large intestine (ascending colon), and appendix

Hindgut

Rest of the large intestine/bowel (transverse and descending colon) and rectum

Symptoms

GEP-NETs can cause a wide variety of symptoms that are often mistaken as other conditions.¹² This, coupled with the fact that some GEP-NETs may not produce any symptoms at all, means these tumors can go undetected for years before a final diagnosis is made.¹²

Symptoms can be caused by the physical presence of the tumor inside the body, or the release of too many hormones from functioning tumors.⁸ For people with functioning GEP-NETs, the type of hormone produced by the tumor can influence the symptoms they experience.¹³ Symptoms may also be dependent on where the tumor appears in the body.¹⁴

Common symptoms due to the physical presence of the tumor¹⁴

Potential symptoms caused by the production of specific hormones^8,13,15,16

This list is non-exhaustive. If you are experiencing any symptoms that are new or affecting you, contact your clinical team for help with symptom relief and advice.

Diagnosing and monitoring GEP-NETs

There are a variety of different ways to detect the presence of the tumor and monitor it closely to check if it grows, changes or spreads.¹⁷ You may have already experienced one or many of the tests discussed below. If you have any questions about tests or monitoring and diagnosis, reach out to your doctor or clinical team for information specific to your situation.

This list is not exhaustive, and you may have encountered other methods to monitor GEP-NETs. If needed, reach out to your clinical team for information specific to your situation.
*Inform your doctor or clinical team if you feel unwell after taking the contrast fluid or tracer.^19,21
^†The injection only contains a small amount of radiation and is usually not harmful.²²

This list is not exhaustive, and you may have encountered other methods to monitor GEP-NETs. If needed, reach out to your clinical team for information specific to your situation.

Grading and staging of GEP-NETs

Tumors are given a grade to determine how fast they are growing. Grading is based on the number of cells within the tumor that are actively dividing and replicating, meaning making new cells.²⁸ To grade your tumor, a member of your clinical team will take a sample of tissue from your body so the tumor cells can be examined.⁴ There are different ways of doing this,¹³ and your doctor may be able to discuss the different options with you.

A member of the clinical team will look at the tissue sample to determine the tumor grade. If fewer cells are dividing and replicating it means the tumor growth is slower. On the other hand, the quicker the tumor cells divide, the higher the grade of the tumor will be.²⁸ GEP-NETs are generally slow-growing,^29,30 however some tumors can progress into higher grades^30,31 or spread to other organs.¹⁰

Grading system for GEP-NETs^7,32

Grade 1. Low. Cells divide at a low rate and grow slowly. Grade 2. Intermediate. Cells divide at an intermediate rate. Grade 3. High. Cells divide at a high rate and grow quickly.

These tumor cells can also be described as well differentiated or poorly differentiated depending on how they appear compared to healthy cells:²⁸

Well differentiated cells look more like healthy cells
Poorly differentiated cells look less like healthy cells

Staging is a term that describes where the tumor is located, if and where it has spread to and whether it is affecting other parts of the body. The Tumor, Node, Metastasis (TNM) system will commonly be used by your clinical team where the results of the following questions are combined to determine the stage of your cancer:³³

Tumor (T): How large is the primary tumor? Where is it located?
Node (N): Has the tumor spread to the lymph nodes? If so, where and how many?
Metastasis (M): Has the cancer spread to other parts of the body? If so, where and how much?

The tumor’s grade, alongside other factors, such as stage and functionality, can help inform decision-making about treatment.⁴ Understanding how your tumor is behaving can help your clinical team explain this to you and discuss recommended treatment options with you.²⁸

Talking to people about your GEP-NET diagnosis

You may find it helpful to tell people close to you so they’re aware and can best support you. Sharing the information about your GEP-NET diagnosis can be especially difficult as it is a rare disease and therefore not well-known to the general population. Communicating your diagnosis could also be an uncomfortable experience depending on how people react to it. Here are some considerations if you’re finding it difficult with telling your friends, family, or others.

How do you want to tell them?

You could tell people face-to-face, over the phone, via email, or with a letter. Take some time to think about who you’d want to tell. It is advisable to tell people that you think can offer you positive support. Sharing the information with your loved ones will help you feel accompanied, and it will be easier for them to support you if they are involved in your disease journey.³⁴

How many people do you want to tell?

You may want to tell a few people close to you and ask others to help convey the information.³⁴ Remember that your GEP-NET diagnosis is part of your life and who you share this information with is a personal decision.³⁵ If required, contact a local patient organization to discuss potential ways of communicating your condition to your employer and work colleagues.

Dealing with emotions

It’s important to realize that your disease might not be visible to other people. Therefore, telling people may be an emotional and overwhelming experience for you and those you tell. You may want to break up the news with unrelated conversation before returning to the topic. It’s okay to feel overwhelmed when talking to people, and take breaks when you need it. It’s okay to end the discussion until you’re ready to pick it up again and continue.³⁴

Dealing with reactions

If you feel comfortable enough, try not to downplay, hide, or omit any information that you feel may affect the people you are telling. They could be able to support you better if they understand the true nature of your diagnosis.³⁴

It’s important to realize that you are not responsible for how people react to the news, and it’s okay to feel awkward when receiving emotional responses. If you’re overwhelmed at any point, you can pause and take a breath, or be emotional alongside those you are telling.³⁴

If you would find it useful to have specialized advice before or after telling anyone, feel free to contact your clinical team or patient organization. They may be able to refer you to a psycho-oncologist or dedicated counselling/psychotherapy team for support depending on availability in your area.

For additional support, the International Neuroendocrine Cancer Alliance (INCA) can help you find your nearest support groups and societies (https://incalliance.org/world-net-community-directory/).⁵

Working or studying with GEP-NETs

You may want to continue working, studying, or any regular commitment after being diagnosed with a GEP-NET or take a break from them. It is important and positive that you honestly value how you feel and that you avoid putting too much pressure on yourself.

If you’re continuing to work or study, you may want to discuss the best way to do this with your clinical team. You may also want to make a list of things that would help while working to discuss with your workplace. This could be things as simple as increased number of breaks and getting time off for treatment and checkups.³⁶

Some educational institutions can support you as you study, they may also be able to defer your study so you can continue when you feel you’re able.³⁷

For specific advice on local regulations and labor rights, visit https://incalliance.org/world-net-community-directory/ to find a patient organization that may be able to assist you.⁵

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Dittrich RP and De Jesus O. Gallium Scan. Available at: https://www.ncbi.nlm.nih.gov/books/NBK567748/. Accessed: October 2021. 26. Sansone A, Lauretta R, Vottari S, et al. Specific and Non-Specific Biomarkers in Neuroendocrine Gastroenteropancreatic Tumors. Cancers (Basel). 2019; 11(8): 1113. 27. Cancer Research UK. Urine tests for neuroendocrine tumors (NETs). Available at: https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/getting-diagnosed/tests/urine-test. Accessed: October 2021. 28. Cancer.Net. Neuroendocrine Tumors: Grades. Available at: https://www.cancer.net/cancer-types/neuroendocrine-tumors/grades. Accessed: October 2021. 29. Fazio N. Watch and wait policy in advanced neuroendocrine tumors: What does it mean? World J Clin Oncol 2017; 8(2): 96-99. 30. Uri I and Grozinsky-Glasberg S. Current treatment strategies for patients with advanced gastroenteropancreatic neuroendocrine tumors (GEP-NETs). Clin Diabetes Endocrinol. 2018; 4: 16. 31. Keck KJ, Choi A, Maxwell JE et al. Increased Grade in Neuroendocrine Tumor Metastases Negatively Impacts Survival. Ann Surg Oncol. 2017; 24(8): 2206-2212. 32. Nagtegaal ID, Odze RD, Klimstra D et al for the WHO Classification of Tumours Editorial Board. Histopathology 2020; 76: 182-188. 33. Cancer.Net. Neuroendocrine Tumor of the Gastrointestinal Tract: Stages and Grades. Available at: https://www.cancer.net/cancer-types/neuroendocrine-tumor-gastrointestinal-tract/stages-and-grades. Accessed: October 2021. 34. Macmillan. How to tell people you have cancer. Available at: https://www.macmillan.org.uk/cancer-information-and-support/diagnosis/talking-about-cancer/how-to-tell-people-you-have-cancer. Accessed: October 2021. 35. Survivorship. An ESMO-ECPC guide for patients in collaboration with IPOS. Available at: https://www.ipos-society.org/patients/resources. Accessed: October 2021. 36. Young Lives vs Cancer. How do I tell my employer? 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Recently diagnosed with a gastroenteropancreatic neuroendocrine tumor (GEP-NET)