Neuroendocrine tumours

About

Neuroendocrine tumours (NETs) are a type of cancerous tumour that arise from cells of the endocrine and nervous systems. They can occur anywhere in the body, but are most commonly found in the digestive system, respiratory system, and endocrine glands. NETs are usually very slow-growing and can be characterized by specific clinical and biological features. Symptoms vary depending on the area of the body affected and can include abnormal growth hormone production, diabetes, diarrhea, abdominal pain, and weight gain. Treatment may involve surgery, radiation, medication, and/or chemotherapy.

Symptoms

The symptoms of neuroendocrine tumors are highly variable and depend on the location, type, and size of the tumor. Some of the common symptoms include abdominal pain, vomiting, weight loss, fever, and fatigue. Additionally, neuroendocrine tumors can cause increased levels of hormones such as serotonin, ACTH, and gastrin, which can cause specific symptoms such as flushing, diarrhea, and hypoglycemia. Other symptoms experienced can include headache, bone pain, jaundice, chest pain, respiratory difficulty, and night sweats.

Causes

The exact cause of most neuroendocrine tumours is unknown, but certain factors may increase the risk of developing them.

These factors include a family history of the tumours, certain genetic conditions such as multiple endocrine neoplasia type 1 and von Hippel-Lindau syndrome, certain autoimmune diseases such as Sjogren’s syndrome, and exposure to certain environmental toxins. Age is also thought to play a role in certain types of these tumours.

Risk factors

Factors that may increase the risk of developing neuroendocrine tumors include:

  • Family history of neuroendocrine tumors
  • Certain inherited genetic syndromes, including multiple endocrine neoplasia
  • Exposure to environmental toxins, including vinyl chloride
  • Advanced age
  • Certain types of inflammatory bowel disease
  • Chronic pancreatitis
  • Gender (males are more likely to develop neuroendocrine tumors than females)
  • Obesity
  • Smoking

Diagnosis

Neuroendocrine tumours can be diagnosed through a variety of tests. Doctors may start by doing a physical exam and taking a patient’s complete medical history. Imaging tests such as CT scans, MRIs, and ultrasound may be done to look for the tumour and see how far it has spread. A biopsy or examination of cells or tissue taken from the tumour itself is also a common diagnostic tool for neuroendocrine tumours. Blood tests can check for certain hormones or chemicals in the body. A nuclear medicine scan, also called a somatostatin receptor scan, can detect somatostatin receptors which are produced by some neuroendocrine tumours. Finally, doctors may use laparoscopy or endoscopy, which are more invasive techniques to view the tumour.

Sub-types

Neuroendocrine tumors (NETs) are a diverse group of tumors that originate in the specialized cells of the endocrine and nervous systems. They are typically divided into four distinct subtypes based on their location and behavior: carcinoid tumors, pancreatic NETs, small cell lung cancer, and pheochromocytomas.

Carcinoid tumors are typically slow-growing tumors that can form in the digestive system, lungs, or other organs and secrete hormones. Pancreatic NETs typically form in the pancreas and can be benign or malignant. Small cell lung cancer forms in the lung and is typically an aggressive and rapidly progressing cancer. Pheochromocytomas form in the adrenal glands and secrete hormones that can cause high blood pressure, nausea, and sweating.

Treatments

The treatment options for Neuroendocrine tumours depend on the size, type and location of the tumour. Generally, surgery, radiation therapy, and/or chemotherapy may be used.

Surgery, the most common treatment option, is typically used to remove the tumour and address any related symptoms. In some cases, a minimally invasive procedure may be used to remove the tumour, such as laparoscopic surgery.

Radiation therapy may also be used to shrink the tumour, relieve symptoms and slow the growth of the tumour. It can be used alone or in combination with other treatments such as chemotherapy or surgery.

Chemotherapy is used to treat Neuroendocrine tumours that are more advanced. It is typically given as an intravenous (IV) infusion, although some drugs can be taken orally. It works to block the growth of cancer cells and may also be used in conjunction with surgery or radiation therapy.

In addition to these techniques, other treatments may be used to address symptoms of the tumour, such as pain management and nausea control. Some Neuroendocrine tumour patients may also be offered immunotherapy, hormone therapy, or targeted therapy.

The treatment plan for Neuroendocrine tumours will vary depending on the individual situation and should be discussed with a medical professional.

Prevention

There are a few steps that can be taken to reduce the risk of neuroendocrine tumours:

  1. Maintain a healthy lifestyle: Eating a balanced diet, exercising regularly and avoiding smoking can help reduce the risk of neurological tumours.
  2. Avoid prolonged exposure to certain chemicals: Prolonged exposure to certain chemicals, such as cadmium, may increase the risk of developing a neuroendocrine tumour.
  3. Get screened: Regular screenings for neuroendocrine tumours can help to diagnose and treat them early.
  4. Pay attention to symptoms: Watch out for symptoms such as abdominal pain, changes in mood and energy level, changes in weight, fatigue, and digestive problems. If any of these symptoms appear, seek medical attention quickly.

Gender differences?

Yes, there are gender-specific differences in the presentation and management of Neuroendocrine tumours. Neuroendocrine tumours are often more aggressive in men than in women, with a higher mortality rate in male patients. Women with Neuroendocrine tumours tend to be diagnosed at a younger age and with smaller tumours. Additionally, women with Neuroendocrine tumours often present with different symptoms than men, including abdominal pain, constipation, and bloating.

In terms of management, the treatment for Neuroendocrine tumours depends on the stage and size of the tumour, as well as the patient’s overall health. For example, women may be recommended to have a hysterectomy in order to remove a tumour, while this may not be an option for men. Women may also be offered more conservative treatments due to their lower risk of aggressive disease progression. Additionally, women may require different hormonal therapies to men, due to the difference in hormones between the sexes.

Nutrition

A healthy, balanced diet is an important part of managing neuroendocrine tumours. Eating a variety of nutritious foods can help to reduce symptoms, such as fatigue, maintain strength and energy levels, optimize organ function, and support overall wellbeing. A diet rich in whole grains, fruits, vegetables, and lean proteins can help to keep the body functioning optimally and to reduce symptoms related to neuroendocrine tumours. Additionally, limiting foods that are high in sugar and processed carbohydrates can help to reduce symptoms such as bloating and abdominal discomfort. Hydration is also very important, and water should be consumed throughout the day to help the body stay hydrated and functioning at its best. Supplementation with a multivitamin, probiotics, and omega-3 fatty acids may be beneficial as well, depending on the individual’s health needs. Ultimately, it is important to speak with a doctor and/or registered dietitian to develop an individualized nutrition plan for managing neuroendocrine tumours.

Physical Activity

Physical activity can have a positive effect on neuroendocrine tumours. Regular aerobic activities like walking, jogging, or swimming can help reduce stress, increase endorphins, and help you manage your symptoms better. In addition, physical activity can help you maintain a healthy weight, which is important for neuroendocrine tumour management. Additionally, exercise can help reduce the risk of complications such as cardiovascular and metabolic diseases that can be associated with neuroendocrine tumours. However, it is important to talk to your doctor before starting any exercise program in order to ensure that it is safe and appropriate for you.

Further Reading

  1. https://rarediseases.info.nih.gov/diseases/13445/neuroendocrine-tumor/
  2. https://www.cancerresearchuk.org/about-cancer/neuroendocrine-tumours-nets/what-are-nets
  3. https://fg.bmj.com/content/13/1/50
  4. https://www.mayoclinic.org/diseases-conditions/neuroendocrine-tumors/symptoms-causes/syc-20354132
  5. https://bmccancer.biomedcentral.com/articles/10.1186/s12885-019-6412-8

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