16 March 2018

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Explained What is neuroendocrine tumour symptoms treatment stages

Explained What is neuroendocrine tumour symptoms treatment stages
Meaning -
The neuroendocrine system - The neuroendocrine system is made up of nerve and gland cells. It makes hormones and releases them into the bloodstream.

Neuro means nerve and endocrine means the cells of the endocrine system. The endocrine system is a network of glands and organs in the body that produce hormones. It's also called the hormone system.

Generally speaking, hormones control how our bodies work. This includes our growth and development, how we respond to changes such as stress, and many other things.
There are neuroendocrine cells in most organs of the body.

Neuroendocrine cells have different functions depending on where they are in the body.
 For example, neuroendocrine cells in the gut make hormones to control:
1-the release of digestive juices into the gut
2-the muscles that move food through the bowel

Neuroendocrine cells in the lung release hormones that control the flow of air and blood in the lungs.
Neuroendocrine tumours (NETs) are rare tumours that develop in cells of the neuroendocrine system. There are a number of different types of neuroendocrine tumour. The type you have depends on the particular cells that are affected.

Most neuroendocrine tumours usually develop slowly over a number of years. Many people don’t have symptoms at first. It's not unusual for people to find that a neuroendocrine cancer has already spread to another part of the body when they are diagnosed.

Other terms used for neuroendocrine tumours include:
1-gastroenteropancreatic neuroendocrine tumours (GEP NETs for short) – tumours in the gut or pancreas
2-pancreatic neuroendocrine tumours (pNETs) – tumours that develop in the pancreas
3-gastrointestinal neuroendocrine tumours (GI NETs) – tumours that develop in the bowel, stomach or food pipe (oesophagus)
4-functioning neuroendocrine tumours (F-NETs)
5-non functioning neuroendocrine tumours (NF-NETs)
6-carcinoid tumour

neuroendocrine tumor - A tumor that forms from cells that release hormones into the blood in response to a signal from the nervous system. Neuroendocrine tumors may make higher-than-normal amounts of hormones, which can cause many different symptoms. These tumors may be benign (not cancer) or malignant (cancer). Some examples of neuroendocrine tumors are carcinoid tumors, islet cell tumors, medullary thyroid cancer, pheochromocytomas, neuroendocrine carcinoma of the skin (Merkel cell cancer), small cell lung cancer, and large cell neuroendocrine carcinoma (a rare type of lung cancer).

Neuroendocrine Tumors (NETs) are cancer, which means they can spread to other parts of your body. Many of these tumors also make hormones of their own, which can give you certain symptoms. Other kinds of neuroendocrine tumors are benign, which means they don't move from their original spot.

Most neuroendocrine tumors grow slowly -- over years, not months -- compared with other types of tumors. Often, doctors can remove or shrink them with different treatments. Other therapies can make your symptoms better.

There are many types of NETs. They're usually named after the type of cell where they grow, or the hormone they make.

Types of neuroendocrine tumour
There are a number of different types of NET. They are generally called after the organ or type of cell they develop in.
NETs most often develop in the gut or pancreas. These tumours are sometimes grouped together and called gastroenteropancreatic neuroendocrine tumours or GEP NETs for short.
NETs in the gut
NETs of the gut are most often carcinoid tumours. They might also just be called carcinoid.
NETs in the pancreas
NETs that develop in the pancreas are also called endocrine tumours of the pancreas.

These are all rare types of tumour and include:
1-insulinomas, which produce insulin

2-gastrinomas, which produce gastrin (a hormone that helps digest food)

3-glucagonomas, which produce glucagon (a hormone that helps to raise blood sugar levels)

4-VIPomas, which produce vasoactive intestinal peptide (VIP for short) that helps digestion and a number of other body processes

5-somatostatinoma, which produces the hormone somatostatin that helps with digestion

Some of these tumours can also develop outside the pancreas. For example, gastrinomas have been reported in the medical literature to develop in the ovaries, kidneys, stomach and liver.
NETs in the lung NETs that develop in the lung are usually carcinoid tumours.

Other rare types

There are a number of other even rarer types of NET including those that develop in the:

1-thyroid gland – medullary thyroid tumours

2-skin – Merkel cell cancer

3-pituitary gland – a small gland in the brain

4-parathyroid gland – small glands attached to the thyroid gland

5-adrenal glands – usually called adrenomedullary NETs

Carcinoid tumors can form in many areas of your body, but they're most common in the cells of the digestive system -- the stomach, small intestines, appendix, and rectum. They can also form in the lungs or a small organ behind the breast bone called the thymus. More rarely, they grow in the pancreas, kidneys, ovaries, or testicles.

A neuroendocrine tumour is a rare tumour that can develop in many different organs of the body. It affects the cells that release hormones into the bloodstream (neuroendocrine cells).
Neuroendocrine tumours can be cancerous (malignant) or non-cancerous (benign). They often grow slowly, but not always.

These tumors can release different types of hormones, which can affect how you feel. Doctors call these groups of symptoms carcinoid syndrome.

Pancreatic NETs grow in your pancreas. There are a few kinds of them:
Insulinomas are the most common type. Their cells make insulin, the hormone that controls blood sugar levels. Most of the time, they're not cancerous.

Glucagonomas make glucagon, a hormone that raises your blood sugar level. About half of them are cancerous, and they often spread to other parts of your body.

Gastrinomas make the hormone gastrin, which helps you digest food. These tumors can happen if you have a rare disorder called Zollinger-Ellison syndrome. About half of these gastrinomas are cancerous, and they often spread easily in the body.

Somatostatinomas make too much of a chemical called somatostatin that affects how your body makes other hormones.

VIPomas make a hormone that triggers the release of other hormones, called vasoactive intestinal peptides (VIP). Most VIPomas are cancerous.

Some other types of NETs include:
Medullary carcinoma. It shows up in your thyroid gland, an organ that's on the base of your neck. This tumor grows in cells that make a hormone that affects the levels of calcium in your body.

Pheochromocytoma. This grows in cells of your adrenal glands, which sit above your kidneys. It makes the hormones adrenaline and noradrenaline, which can increase your heart rate and blood pressure. Usually these tumors are not cancerous.

Symptoms of neuroendocrine tumours -
The symptoms of a neuroendocrine tumour depend on where in the body it is and what hormones it produces.
For example, a tumour in the digestive system (gastrointestinal neuroendocrine tumour) may cause diarrhoea, constipation or tummy pains.
A tumour in the lung (pulmonary neuroendocrine tumour) may cause wheezing or a persistent cough.
Some tumours may lead to abnormally large amounts of hormones being released into the bloodstream. These are known as "functioning tumours" and can cause symptoms such as diarrhoea, flushing, cramps, wheezing, low blood sugar (hypoglycaemia), changes in blood pressure and heart problems.
Symptoms depend on the type you have and where it is in your body.
With a pancreatic NET, you might have following symptoms.
1-Blurred or double vision
5-A fast heartbeat
7-Hunger that's stronger than usual
10-Stomach pain
13-Weight loss without trying

Carcinoid tumors can cause:
2-Red, warm, itchy skin, often on your face and neck
4-Pain in your chest
5-Stomach pain
6-Feeling tired or sick
7-Trouble breathing
8-Weight gain or loss without trying

Causes of neuroendocrine tumours  -
The cause of neuroendocrine tumours isn't fully understood. However, your chance of developing one is increased if you have one of the following inherited conditions:
1-multiple endocrine neoplasia type 1 (MEN1)
2-neurofibromatosis type 1
3-Von Hippel-Lindau syndrome (VHL)

Multiple endocrine neoplasia type 1. This causes tumors to grow in the pancreas and other organs.

Neurofibromatosis type 1. This can cause tumors in your adrenal glands.

Von Hippel-Lindau syndrome. It makes tumors and fluid-filled sacs form in many parts of your body.

Research suggests your risk of developing a neuroendocrine tumour is slightly increased if your father, mother, brother or sister has had any type of cancer.
Many tests can be used to diagnose neuroendocrine tumours, including blood tests, urine tests, scans and a biopsy (where a small tissue sample is taken for closer examination)

Stages of neuroendocrine tumors
Identifying the stage of disease is an important step in planning the appropriate treatment for neuroendocrine tumors. NETs may:
1-Be contained in a particular area of the body (localized)
2-Have spread to nearby tissues or lymph nodes (regional)
3-Have spread throughout the body (metastatic)

More than 50 percent of NETs have already spread to other parts of the body by the time they are diagnosed. NETs metastasize most often to the liver, peritoneal cavity or bone.
Neuroendocrine tumors are staged according to the TNM staging system: tumor (T), node (N), metastasis (M). The World Health Organization (WHO) classifies neuroendocrine tumors according to the malignant potential of the tumor:
1-Well-differentiated neuroendocrine tumors (grade 1 and 2)
2-Poorly-differentiated neuroendocrine tumors (grade 3)

Treatment for neuroendocrine tumours -
Doctors can treat NETs with surgery, radiation, chemotherapy, and drugs. The treatment you get will depend on:
1-What kind of tumor you have and how many there are
2-Whether it's cancerous
3-If it has spread to other parts of your body

How a neuroendocrine tumour is treated will depend on your individual circumstances, such as:
1-where the tumour is
2-how advanced your condition is
3-your overall health  

Unfortunately, many people are only diagnosed after the tumour has spread to other parts of the body.
However, it may still be possible to use surgery to remove the tumour. If surgery can't completely cure your condition, it may be used to help reduce and manage any symptoms.
Medication called somatostatin analogues, such as octreotide or lanreotide, may be recommended. They stop your body producing too many hormones, and can reduce the severity of your symptoms and slow the condition's progression. You'll have an injection, usually once every 28 days.

It may also be possible to shrink the tumour or stop it growing using treatments that block its blood supply (embolisation), or using chemotherapy, radiotherapy or radiofrequency ablation (where heat is used to destroy the cells).

There are also two treatments – everolimus and sunitinib – that can be used to treat some neuroendocrine tumours in adults if:
1-they can't be fully removed using surgery
2-they've spread
3-the symptoms are getting worse

Everolimus is taken as a tablet, and sunitinib is taken as a capsule.

Surgery. It's one of the most common treatments for many NETs. It can completely remove some tumors, especially those that aren't cancer and haven't spread.
A surgeon might be able to take out just the tumor. Or he may remove part or all of the organs that have a NET, like the pancreas, stomach, or liver.
Doctors can also use other kinds of surgery for people who can't have a traditional operation or who have many, small tumors.
In one type, called radiofrequency ablation, your doctor will put a probe into the tumor that gives off high-energy radio waves, which kills cancer cells in a certain area.
Another type, called cryosurgery, sends extreme cold directly to a tumor with a thin, hollow tube. For these operations, your doctor might use MRI scans or ultrasounds to guide where the probe should go.

Hormone therapy. This is a common treatment for carcinoid NETs. It uses a man-made version of the hormone somatostatin -- usually lanreotide (Somatuline Depot), octreotide (Sandostatin), or pasireotide (Signifor) -- which you get through an injection. These drugs keep the tumor from making hormones that can cause diarrhea and other carcinoid syndrome symptoms. They might also shrink the tumor.

Radiation. This method uses high-energy X-rays to kill cancer cells. You might get this treatment if your tumor has spread or if it's in a place that doctors can't reach with surgery.
Chemotherapy. It uses drugs to kill cancer cells or to stop them from spreading. You take them by mouth, or a doctor injects them into one of your veins. You might take a single medicine or a mix of different ones for a few weeks.

Embolization therapy. This can treat NETs that spread to your liver that doctors can't remove with surgery. The goal is to block the blood flow that helps them thrive.
In a hospital, your doctor will put a thin, flexible tube called a catheter into the artery that leads to the liver. Then, he'll inject a substance to plug up the artery. You might also get chemotherapy or radiation during the procedure.

Targeted therapy. It uses drugs that attack certain genes or proteins on tumor cells to kill cancer. This treatment limits damage to the healthy cells in your body, which can happen with radiation or chemotherapy.

NETs can make it hard to stay at a healthy weight, so focus on eating the right types of foods to get enough nutrition.
1-Get extra protein from fish, eggs, cheese, and beans.
2-If you feel sick to your stomach, try eating smaller meals more often, instead of three big ones. Ginger ale might help calm your stomach.
3-Avoid high-fat foods, sweets, and sugary drinks.

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