Neuroendocrine tumors or NETs are cancers present in cells that are similar to hormone-producing cells as well as nerve cells, known as neuroendocrine cells, which are present throughout the body. Neuroendocrine tumors are rare and may occur anywhere in the body. The tumor occurs in the pancreas, rectum, appendix, small intestine, lungs, and other adrenal glands.
Sometimes, NETs are complex to diagnose. Thus, it is good to take expert advice for treating the tumor. The neuroendocrine tumors fall under different categories. Some multiply quickly while some grow slowly. Some tumors produce hormones [functional NETs] while others do not release excess hormones [nonfunctional NETs].
Before starting the treatment, your doctor will diagnose the type of tumor, where it is present, whether it produces less or excess hormone, how severe it is, and whether it has spread to other body parts.
What are the types of NETs?
NETs can form anywhere in the body where you have those, and they are often named based on where they grow. These include:
● Carcinoid Tumors
Carcinoid tumors occur digestive system, including the small intestine, colon, and rectum. Other areas include lungs, ovaries, testicles (quite rare), pancreas, etc. Such tumors grow slowly. Carcinoid tumors often do not cause any sign and symptom until late into the disease. Carcinoid tumors might produce and release hormones into your body, which can cause signs and symptoms like skin flushing or diarrhea.
Generally, treatment for carcinoid tumors includes surgery and may include medicines.
● Pancreatic Neuroendocrine Tumors
These originate in the islet cells of the pancreas. Your doctor may describe these tumors as ‘functional’ or ‘non-functional’.
Functional tumors produce excessive similar hormones that lead to symptoms. These hormones control various actions of the body like digestion, blood sugar levels, and heart function.
For example, glucagonomas release glucagon in excess. Similarly, insulinomas release excess insulin, lowering blood sugar levels. Gastrinomas release excessive gastrin, leading to ulcers.
It is a rare tumor that occurs in the adrenal glands which are located above the kidneys.
It releases adrenaline and noradrenaline, which controls heart rate, sugar level, and blood pressure. Mostly, pheochromocytomas are not cancerous. However, the tumor may release hormones that result in heart strokes or heart attacks.
● Merkel cell carcinoma
It is a type of skin cancer. It originates in the skin exposed to the sunlight. It can be in the neck, arms, legs, or head.
What are the Common Symptoms Related to Neuroendocrine Tumors?
The symptoms you experience depend on the nature of the tumor and its origin.
Symptoms of Neuroendocrine Tumors (NETs).
Signs and symptoms may include:
- A growing lump that can be felt under the skin
- Pain from a growing tumor
- Feeling tired unusually
- Losing weight without even trying
Neuroendocrine tumors which produce excessive hormones (functional tumors) may cause:
- Skin flushing
- Increased thirst
- Frequent urination
How are Neuroendocrine Tumors Diagnosed?
Your doctor will conduct a physical examination. They may ask questions related to your health, pain, appetite, weight loss, fatigue, rashes in the skin (if any), medical history, etc.
Further tests include:
- Blood and urine tests. This test is done to assess the hormone levels in the body.
- Imaging tests. You may underdo some imaging tests like MRI, ultrasound and and CT, to create pictures of the tumor. For neuroendocrine tumors, sometimes pictures are created using PET (positron emission tomography) with a radioactive tracer that is injected into your vein.
- Biopsy (procedures to remove a sample of cells for testing). To collect the cells, your doctor may insert a thin, long tube (with a light and camera attached to it on the other end) into your rectum (colonoscopy), your esophagus (endoscopy), or your lungs (bronchoscopy), depending on your condition and situation. Sometimes, collecting a tissue sample needs surgery.
What are the Treatment Options for Neuroendocrine Tumor?
Once you have been diagnosed with NET, your doctor will make a comprehensive treatment plan based on your unique needs. The treatment depends on factors like age, the severity of NET, etc.
Some treatment methods are:
Surgery is the foremost approach for the patient diagnosed with NETs. The objective of this method is to remove the tumor completely.
● Medical oncology
Depending on the NET, treatment methods like chemotherapy, targeted therapy, or hormone therapy are used to manage different NETS. Some targeted therapies include everolimus and sunitinib. These therapies are designed for pancreatic NETs.
● Radiation therapy
This therapy makes use of powerful energy beams like X-rays and protons, to kill cancer tumor cells. Certain types of neuroendocrine tumors may respond to radiation therapy. This therapy might be suggested if surgery is not an option.
● Medicines to control excess hormones
If your neuroendocrine tumor releases excessive hormones, your treating physician may recommend medicines to control your signs and symptoms.
When Should You See a Doctor?
Do not delay. Consult your doctor immediately if you experience any of the symptoms given above. Your doctor will prescribe the right medications based on the tumor stage.
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What are the Underlying Causes of Neuroendocrine Tumors?
The actual cause of the disease is still unidentified. Tumors grow when neuroendocrine cells mutate in their DNA. These changes further lead to multiplication resulting in a tumor.
What are the Risks Related to Neuroendocrine Tumors?
The risk is maximum in people who inherit genetic syndromes. Examples:
- Multiple endocrine neoplasia, type 1 (MEN 1)
- Multiple endocrine neoplasia, type 2 (MEN 2)
- Von Hippel-Lindau disease
- Tuberous sclerosis