WORLD NEUROENDOCRINE TUMOR (NET) AWARENESS DAY

Neuroendocrine tumors (NETs) are rare tumors that start in neuroendocrine cells. These tumors may also be referred to as neuroendocrine carcinomas (NECsor neuroendocrine neoplasms (NENs).¹

Neuro means nerve and endocrine refer to the cells of the endocrine system. The endocrine system is a network of glands and organs in the body that make hormones. It is also called the hormone system. There are neuroendocrine cells in most organs of the body, including the food pipe (esophagus), stomach, lungs, small and large bowel, pancreas, and liver.^{1, 2}

Neuroendocrine cells are scattered throughout the body (figure). There are various forms and subtypes of NET, and some of its symptoms could be mistaken for those of other, less severe illnesses. NETs can begin in several locations across the body. Like all malignancies, NETs are given their name based on where they first appear. For instance, a lung NET is a NET that originates in the lung. The cancer is primary. Cancer that has spread to an additional area of the body is referred to as secondary cancer. Early diagnosis and treatment continue to increase the number of people living for years with NET.^{1, 2}

There are main types of Neuroendocrine tumors:²

• Adrenal cancer
• Carcinoid tumors
• Merkel cell carcinoma
• Pancreatic neuroendocrine tumors
• Paraganglioma
• Pheochromocytoma

Neuroendocrine tumors symptoms and signs: ³

Depending on the type of NET, a person may experience the following tumor-related symptoms or signs:

• Fatigue
• Loss of appetite
• Unexplained weight loss
• Persistent pain in a specific area
• Thickening or a lump in any part of the body
• Changes in bowel or bladder habits
• Jaundice, which is the yellowing of the skin and whites of the eyes
• Unusual bleeding or discharge
• Facial flushing, usually without sweating
• Hyperglycemia and Hypoglycemia
• Ulcer disease
• Skin rash
• Anxiety

Risk factors for NET: ⁴

• Age: A person of any age can develop a NET. Children rarely develop NETs.
  
  
• Gender: NETs are slightly more commonly seen in women than in men.
  
  
• Environment and diet: There are no known connections between the environment, what a person eats, and the risk of developing a NET.
  
  
• Race/ethnicity: In general, NETs are more common in white people than in Black people. However, the location of where the NET begins varies considerably by race.
  
  
• Inherited syndromes: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition associated with certain types of NETs, including lung NETs, GI tract NETs, and pancreatic NETs. Other hereditary conditions related to NETs include Von Hippel-Lindau syndrome, neurofibromatosis type 1, multiple endocrine neoplasia type 2 (MEN2), and tuberous sclerosis complex.
  
  
• Other medical conditions: Certain diseases may put people at greater risk of developing specific types of NETs.
  
  

Treatment for NET:⁵

The treatment options for neuroendocrine tumors will depend on the type of tumor, its location, and whether you’re experiencing signs and symptoms of excess hormones produced by the tumor. Some neuroendocrine tumor treatment options might include:

• Surgery: It is used to remove the tumor. When possible, surgeons work to remove the entire tumor and some of the healthy tissue that surrounds it.
  
  
• Chemotherapy: It uses strong drugs to kill tumor cells. It can be given through a vein in the arm or taken as a pill.
  
  
• Targeted drug therapy: Targeted drug treatments focus on specific abnormalities present within tumor cells. By blocking these abnormalities, targeted drug treatments can cause tumor cells to die.
  
  
• Peptide receptor radionuclide therapy (PRRT): PRRT combines a drug that targets cancer cells with a small amount of a radioactive substance. It allows radiation to be delivered directly to the cancer cells.
  
  
• Medications to control excess hormones: If a neuroendocrine tumor releases excess hormones, the doctor might recommend medications to control your signs and symptoms.
  
  
• Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill tumor cells.

Living with the NET

Coping with feelings

Living with a neuroendocrine tumor is difficult (NET). One may experience persistent symptoms that are challenging to manage. When given a cancer diagnosis, a person may experience a range of emotions. One could experience shock and outrage. It’s normal to feel various emotions as you adjust to having cancer. Many different emotions may come and go. You can handle the challenges you’ll face better by getting counseling. It can aid in stress reduction and life quality enhancement. You can get support and assistance by discussing your disease with friends and family.^{7, 8}

Coping physically

NETs and their treatment might cause physical changes. These can be difficult to cope with and might affect how you feel about yourself. The changes one might have to depend on your type of NET and the treatment you have.

Changes caused by the NET itself

It’s possible to experience changes brought on by the NET itself. Changes could include bowel changes or stomach (abdominal) pain or breathing difficulties if a person has a lung NET. Being short of breath or having abdominal pain can have a big impact on how much one can do each day and most individuals find it extremely terrifying to be unable to breathe.

Inform your doctor or a trained nurse about any changes you notice so they can provide you with appropriate therapy. They can also suggest that you seek the help of other medical experts, including a dietician or the symptom control team (palliative care team).^{7, 8}

Changes caused by the hormones made by the tumor

Some NETs make large amounts of hormones and proteins that are released into the bloodstream. This can cause a collection of symptoms called carcinoid syndrome. ⁷

Changes caused by NET treatments

For NETs, surgery is frequently the sole necessary treatment. However, scars and other physical changes brought on by surgery could have an impact on how one may feel about themselves and how one interacts with others, particularly with close family and friends. Other therapies for NETs include routine injections, which might be uncomfortable where they are administered. It’s possible that a person may have to put up with feeling exhausted and lethargic a lot of the time, particularly after therapy or if their NET is advanced.⁷

World NET Cancer Day is an annual event held on the 10^th of November, created to increase awareness of Neuroendocrine (NET) cancers and to provide a voice to the NET community for improved diagnostics, treatments, information, care, and research. World NET Cancer Day is about drawing attention to this uncommon form of cancer and starting a global conversation amongst communities, medical professionals, and governments.⁸

References:

1. Available at: https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/living-with/coping, Assessed on 18^th October 2022.
2. Neuroendocrine Tumors. Available at: https://my.clevelandclinic.org/health/diseases/22006-neuroendocrine-tumors-net. Assessed on 18^th October 2022.
3. Neuroendocrine tumors. Available at: https://www.mayoclinic.org/diseases-conditions/neuroendocrine-tumors/symptoms-causes/syc-20354132. Assessed on 18^th October 2022.
4. Neuroendocrine Tumors: Symptoms and Signs. Available at: https://www.cancer.net/cancer-types/neuroendocrine-tumors/symptoms-and-signs. Assessed on 18^th October 2022.
5. Neuroendocrine Tumors: Risk Factors. Available at: https://www.cancer.net/cancer-types/neuroendocrine-tumors/risk-factors. Assessed on 18^th October 2022.
6. Neuroendocrine tumors. Available at: https://www.mayoclinic.org/diseases-conditions/neuroendocrine-tumors/diagnosis-treatment/drc-20465865#. Assessed on 18^th October 2022.
7. Available at: https://www.cancer.net/cancer-types/neuroendocrine-tumors/coping-with-treatment, Assessed on 18^th October 2022.
8. NET Cancer Day 2022. Available at: https://www.carcinoid.org/event/net-cancer-day-2022/#. Assessed on 18^th October 2022.