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What Is Neuroendocrine Carcinoma Cancer

Neuroendocrine Tumors Metastatic To The Liver

What is the Neuroendocrine System and how do Neuroendocrine Tumours develop?

Ingemar Ihse, M.D., Gert Lindell, M.D., and Sten Tibblin, M.D.


Neuroendocrine tumors are rare neoplasms presenting complex challenges to diagnosis and treatment. Even in case of metastatic spread to the liver there are some important differences in the nature of these tumors compared to gastrointestinal and pancreatic adenocarcinomas. They typically show 1) specific clinical symptoms and signs and a protracted course, 2) production of hormones and or amines, which are responsible for the specific clinical symptoms and signs and act as tumor markers, and 3) tumor growth inhibition and symptom relief may be achieved by specific blocking agents. About one-half of all neuroendocrine tumors are described as non-functioning meaning that the patients do not have hormone-related symptoms.

Like All Cancers Neuroendocrine Cancers Develop When Cells Divide Uncontrollably And Grow Into A Tumour In The Case Of Neuroendocrine Cancers This Happens In Neuroendocrine Cells

What is the Neuroendocrine System and how do Neuroendocrine Tumours develop?

Symptoms Types and Diagnosis of Neuroendocrine Tumours

Current terminology is simply Neuroendocrine Tumours or with the primary location of the tumour added: for example, Lung Neuroendocrine Tumours , bowel Neuroendocrine Tumours or pancreatic Neuroendocrine Tumours . The most common sites for Neuroendocrine Cancers to form are the digestive system and the lungs. The gastro-intestinal tract accounts for 54.1% cases. Gastro-intestinal tract Neuroendocrine Cancer are usually found in the large bowel and appendix , small intestine , pancreas and stomach . Neuroendocrine Cancer can also develop in the lungs .

Over the last two decades there has been a significant increase in the incidence of Neuroendocrine Cancers, with most of them being sporadic . However, in patients with genetic conditions such as Multiple Endocrine Neoplasia , Von HippelLindau disease and neurofibromatosis , there is an increased risk of developing these tumours.

How Do Neuroendocrine Cells Work

Neuroendocrine cells act like traffic cops monitoring busy intersections and telling drivers when to stop and go. Like them, your endocrine cells monitor signals from your nervous system. When they get the signal, your cells tell your endocrine system to release hormones. Hormones are chemicals that coordinate different body functions, let your body know what to do and when to do it.

But your neuroendocrine cells do more than watch signals and trigger hormone flow. They also make and release hormones that control activity in the organ where theyre located. For example, the neuroendocrine cells in your gut make hormones that drive production of digestive juices and coordinate the muscles that move food through your bowels.

Neuroendocrine tumors happen when your endocrine cells begin to divide and multiply uncontrollably, eventually becoming tumors that can affect the organs where the cells are located.

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Types Of Neuroendocrine Lung Cancer

  • Small cell lung cancer is the most common form of neuroendocrine lung cancer.
  • A rare form of neuroendocrine lung cancer is called large cell neuroendocrine carcinoma. This cancer looks and acts like SCLC, except that the cancerous cells themselves are larger, and it is treated in much the same way as SCLC.
  • Atypical carcinoid tumor

Things To Consider About Neuroendocrine Tumor Survival Rates

Neuroendocrine Cancer Awareness Day 2016

While the overall neuroendocrine tumor survival rate can provide a helpful benchmark for physicians, it is of limited value to individual patients for several reasons. Consider that:

  • The data is based on the collective experiences of thousands of people, and the actual outcome for a specific individual can be markedly different from the general average.
  • It is impossible to predict the prognosis of any patient with a neuroendocrine tumor because every person responds differently to treatment.
  • Survival statistics are captured in five-year intervals therefore, they do not account for the most recent medical advances or breakthroughs in treatment approaches.

At Moffitt Cancer Center, each patient with a neuroendocrine tumor is evaluated thoroughly by a multispecialty team of experts who have the expertise necessary to design tailored treatment plans for every type of neuroendocrine cancer. Additionally, we have a surgical team that specializes exclusively in neuroendocrine tumor surgery and performs complex procedures with an unparalleled level of skill and precision.

If youd like to learn more about the neuroendocrine tumor survival rate, the experts at Moffitt can explain how it relates to your unique situation. Call or complete a new patient registration form online. No referrals are required.


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What Causes Neuroendocrine Cancer

In most cases, doctors aren’t certain of any specific cause. Smoking may increase risk, and a small portion of NETs are linked to inherited genetic conditions such as:

  • Multiple endocrine neoplasia Type 1, or MEN1
  • Neurofibromatosis Type 1
  • Von-Hippel Lindau syndrome OHSU offers genetic counseling, testing and risk-assessment services if you have or might have a family history of cancer.

Having Certain Syndromes Can Increase The Risk Of Pancreatic Nets

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer not having risk factors doesn’t mean that you will not get cancer. Talk with your doctor if you think you may be at risk.

Multiple endocrine neoplasia type 1 syndrome is a risk factor for pancreatic NETs.

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What Are Neuroendocrine Tumors

When you first hear that you’ve got a neuroendocrine tumor, you’ll have lots of questions about what it is and how it will affect you. There are quite a few types of this disease, and it can show up in many places in your body.

Your symptoms may depend on where your tumor is growing and what kind it is. Learn as much as you can about your own type of NET, so you can be a confident partner with your doctor when you make decisions on a treatment plan.

While all this is going on, don’t neglect your emotional needs. Your doctor can tell you how to find a support group where you can talk to others who are going through the same things you are. And feel free to open up to your friends and family about how you’re doing. They know you best and can be a huge source of support.

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The first thing you want to find out about your condition is where your tumor is located. NETs grow in cells that make hormones — chemicals that help control different actions in your body, like hair growth, your sex drive, and even your mood. A neuroendocrine tumor can grow in spots like your pancreas, a gland in your belly. It can also happen in your stomach, intestines, or lungs.

If Treatment Does Not Work

Neuroendocrine Tumors An Introduction

Recovery from a NET is not always possible. If the tumor cannot be cured or controlled, the disease may be called advanced or incurable.

This diagnosis is stressful, and for some people, advanced NET is difficult to discuss. However, it is important to have open and honest conversations with your health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.

People who have advanced disease and who are expected to live less than 6 months may want to consider hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.

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Can Net Cancers Be Cured

When caught at an early stage, NET cancers can often be cured with surgery. As with all cancers, if a surgical cure is possible, this should be the first line of treatment. Patients must still be followed for at least ten years to ensure there is no recurrence of the cancer. Unfortunately, many patients are diagnosed later on, when the cancer has spread to other parts of the body. Even when the tumours have spread, the disease and its symptoms can often be controlled for many years. Please check our patient resources and fact sheets for more information.

There are a number of different types of NETs and they all have a slightly different way of presenting themselves, both in terms of symptoms and how they look under a microscope .

The naming of NET tumours is derived from the anatomical area from which they are found in the body and some are named based on the predominant hormone that they secrete.

Are There Grades Of Neuroendocrine Tumors

Yes, healthcare providers use a grading system to create your treatment plan. They consider factors such as how quickly your cancerous cells divide and whether your cancerous cells look more like healthy cells than cancerous cells when viewed under a microscope. Understanding the staging and grading process might be helpful information as you and your provider discuss treatment plans.

What are NETs grades?

  • Grade 1 : These cells divide at a low rate and are growing slowly.
  • Grade 2 : These cells divide at an intermediate rate.
  • Grade 3 : These cells divide at a fast rate and therefore grow quickly.

NETs are also evaluated by degrees of differentiation. Those are:

  • Well-differentiated neuroendocrine tumor: Your cells look more like healthy cells than cancerous cells.
  • Poorly-differentiated neuroendocrine tumor: Your cells look less like healthy cells and more like cancerous cells. Poorly-differentiated cells are then subdivided into small-cell and large-cell tumor classes.

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Certain Factors Affect Prognosis And Treatment Options

Pancreatic NETs can often be cured. The prognosis and treatment options depend on the following:

  • The type of cancer cell.
  • Where the tumor is found in the pancreas.
  • Whether the tumor has spread to more than one place in the pancreas or to other parts of the body.
  • Whether the patient has MEN1 syndrome.
  • The patient’s age and general health.
  • Whether the cancer has just been diagnosed or has recurred .

Different Types Of Pancreatic Nets Have Different Signs And Symptoms

Treatment Options for Pancreatic Neuroendocrine Tumors ...

Signs or symptoms can be caused by the growth of the tumor and/or by hormones the tumor makes or by other conditions. Some tumors may not cause signs or symptoms. Check with your doctor if you have any of these problems.

Signs and symptoms of a non-functional pancreatic NET

A non-functional pancreatic NET may grow for a long time without causing signs or symptoms. It may grow large or spread to other parts of the body before it causes signs or symptoms, such as:

  • Diarrhea.
  • A lump in the abdomen.
  • Pain in the abdomen or back.
  • Yellowing of the skin and whites of the eyes.

Signs and symptoms of a functional pancreatic NET

The signs and symptoms of a functional pancreatic NET depend on the type of hormone being made.

Too much gastrin may cause:

  • Stomach ulcers that keep coming back.
  • Pain in the abdomen, which may spread to the back. The pain may come and go and it may go away after taking an antacid.
  • The flow of stomach contents back into the esophagus .
  • Diarrhea.

Too much insulin may cause:

  • Low blood sugar. This can cause blurred vision, headache, and feeling lightheaded, tired, weak, shaky, nervous, irritable, sweaty, confused, or hungry.
  • Fast heartbeat.

Too much glucagon may cause:

Too much vasoactive intestinal peptide may cause:

Too much somatostatin may cause:

  • High blood sugar. This can cause headaches, frequent urination, dry skin and mouth, or feeling hungry, thirsty, tired, or weak.
  • Diarrhea.
  • Yellowing of the skin and whites of the eyes.
  • Weight loss for no known reason.

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Other Kinds Of Lab Tests Are Used To Check For The Specific Type Of Pancreatic Nets

The following tests and procedures may be used:



  • Fasting serum glucose and insulin test: A test in which a blood sample is checked to measure the amounts of glucose and insulin in the blood. The test is done after the patient has had nothing to eat or drink for at least 24 hours.


  • Fasting serum glucagon test: A test in which a blood sample is checked to measure the amount of glucagon in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours.

Other tumor types

  • VIPoma
  • Serum VIP test: A test in which a blood sample is checked to measure the amount of VIP.
  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual amount of a substance can be a sign of disease. In VIPoma, there is a lower than normal amount of potassium.
  • Stool analysis: A stool sample is checked for a higher than normal sodium and potassium levels.
  • Somatostatinoma
  • Fasting serum somatostatin test: A test in which a blood sample is checked to measure the amount of somatostatin in the blood. The test is done after the patient has had nothing to eat or drink for at least 8 hours.
  • Sorts Of Neuroendocrine Tumor

    The origin level of NET is the cells of the neuroendocrine system. This situation could be indolent or aggressive. Indolent means the NET grows step by step whereas aggressive means NET grows speedily. Generally, the forms of Neuroendocrine Tumors are primarily based on the place of NET. Listed below are some forms of NET.

    • Merkel cell carcinoma
    • Pheochromocytoma of the adrenal gland
    • Adrenal most cancers
    • Thickening or lump in any a part of the physique
    • Modifications in bowel or bladder habits
    • Jaundice

    The affected person ought to research extra about the kind of tumor he/she is affected by. This may assist in alternative ways emotionally in addition to mentally.

    In contrast with different forms of tumors, NET develops slowly- over time. If recognized at an early stage, the docs can shrink or take away NET with the assistance of medicines or therapies.

    As we all know, there are various forms of neuroendocrine tumors, they should be identified at an early stage. Lets focus on the analysis and remedy of neuroendocrine tumors.

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    What Can I Expect If I Have Net

    NETs are sometimes mistaken for other less serious conditions. Your prognosis or expected outcome depends on several factors, including the type of NET you have and whether your tumor has spread. On average, an estimated 77% of people with NETs are alive five years after diagnosis.

    Finishing treatment is a major milestone but not the end of your NET journey. NETs can grow very slowly, so your healthcare provider will want to track your well-being for several years. In some cases, having NETs might be like living with a chronic illness.

    Pancreatic Neuroendocrine Tumors Treatment Patient Version

    Understanding Neuroendocrine Cancer
    On This Page
    • Pancreatic neuroendocrine tumors form in hormone-making cells of the pancreas.
    • Pancreatic NETs may or may not cause signs or symptoms.
    • There are different kinds of functional pancreatic NETs.
    • Having certain syndromes can increase the risk of pancreatic NETs.
    • Different types of pancreatic NETs have different signs and symptoms.
    • Lab tests and imaging tests are used to diagnose pancreatic NETs.
    • Other kinds of lab tests are used to check for the specific type of pancreatic NETs.
    • Certain factors affect prognosis and treatment options.

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    Types Of Neuroendocrine Lung Tumors

    Neuroendocrine cells are specialized cells responsible for repairing epithelial tissues that line the airways, intestines, and other surfaces of the body. They function both as neurons and endocrine cells .

    There are four different types of neuroendocrine tumors of the lungs. A NET can vary widely in terms of its cell type, aggressiveness, response to treatment, and prognosis. The one common feature among them is that they usually develop in the central airways, called the bronchi, located nearer to the middle of the chest.

    Each type of neuroendocrine lung tumor is graded by its severity:

    NET Type

    No Diagnosis For Neuroendocrine Tumors Is The Sameget Personalized Treatment

    Neuroendocrine tumors form in cells that interact with the nervous system or in glands that produce hormones. These cells, called neuroendocrine cells, can be found throughout the body, but NETs are most often found in the abdomen, especially in the gastrointestinal tract. These tumors may also be found in the lungs, pancreas and adrenal glands. Merkel cell cancer, a type of skin cancer, also is considered a neuroendocrine cancer.

    In addition to being rare, NETs are complex and may be difficult to diagnose. Thats why its important to get expert care from a dedicated team with experience and training in treating cancer. Your individualized plan will include evidence-based treatments and technologies to fight neuroendocrine cancer, combined with supportive care services to help reduce side effects and keep you strong in body, mind and spirit.

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    What Are Neuroendocrine Cells

    Neuroendocrine cells are spread throughout the human body, but are mainly found in the small intestine, pancreas, and lung bronchioles. Neuroendocrine cells act like nerve cells in that they receive messages from the nervous system they can also release small molecules that circulate throughout the body and work like hormones. High levels of these hormone-like substances can cause symptoms such as flushing or diarrhea.


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