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Neuroendocrine Cancer (NETs)

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Neuroendocrine tumors, or NETs, are a type of cancer originating in neuroendocrine cells. These cells are unique because they share characteristics with nerve and hormone-producing endocrine cells. While some NETs grow slowly, others can be fast-growing.

NETs can develop in various parts of the body but are most commonly found in the:

  • Appendix
  • Lungs
  • Pancreas
  • Rectum
  • Small intestine

These rare tumors often mimic the symptoms of other common ailments such as asthma, Crohn’s disease, or irritable bowel syndrome.

Although the rates of most common cancers have stabilized over the past 40 years in the United States, the occurrence of NETs has increased six to seven-fold during the same period. More than 170,000 people are currently living with a neuroendocrine tumor, according to Cancer.net.


Your Tumor Board

Your tumor board is a team of doctors and health care providers from different specialties. This team meets regularly to discuss your cancer case and share test results and other information to provide the best possible treatment for you. With their unique perspectives, these team members work together to create an integrated treatment plan customized to your specific needs.

Here's How it Works:

Diagnosis

Your doctor shares the cancer diagnosis with you and talks to you about the next steps to develop a treatment plan unique to you.

Dedicated Multidisciplinary Tumor Board

Specialists such as oncologists, surgeons, pathologists, researchers and others come together to review your cancer case, interpret results and share their recommendations based on their experience and the latest findings in their respective fields.

Personalized Treatment Plan

Through this collaborative team approach, you're ensured the most comprehensive evaluation of your cancer, and a treatment plan is developed for you, which may include participating in clinical trials.

Treatment Begins

Your personalized cancer treatment begins, based on a well-thought-out plan from a diverse team of leading specialists and backed by solid research — all directed toward your best outcome.

The Sylvester Difference

Sylvester Comprehensive Cancer Center offers a dedicated Neuroendocrine Tumors Program, emphasizing research and treatment of these complex cancers, under the leadership of Dr. Aman Chauhan.

Our group of specialists are leading experts in the field of theragnostics, a term coined from "therapy" and "diagnostics." This approach combines diagnostic imaging and therapy to identify and treat various conditions, including neuroendocrine tumors (NETs).

At Sylvester, the focus on clinical trials and research is paramount in shaping the future of neuroendocrine tumor treatment. The facility’s commitment to research means that patients have access to experimental treatments and protocols not widely available.


Types of Neuroendocrine Tumors

NETs vary in their characteristics:

  • Functional NETs: Produce excess hormones and cause symptoms.
  • Non-Functional NETs: Don’t produce enough hormones to cause symptoms, making diagnosis difficult.

They can also be classified by their growth speed:

  • Indolent NETs: Slow-growing and closely resemble normal cells.
  • Aggressive NETs: Fast-growing and resemble cancerous cells.

Regardless of their growth rate, all NETs are cancerous (malignant).

Neuroendocrine tumors are usually classified by their site of origin. Different types of neuroendocrine tumors include:

Gastrointestinal (GI) NETs Page 1

NETs commonly develop in the gastrointestinal tract. Most GI NETs start in the small intestine. They may also develop in the rectum and appendix.

Lung NETs Page 1

Lung NETs develop in the airways of the lungs. They may be central to the main airway of the lungs or peripheral, meaning they develop in the smaller airways toward the outer edges of the lungs.

Pancreatic NETs Page 1

Pancreatic NETs are rare. If they are functional, they may be classified by the type of hormone they produce. These tumors form in hormone-creating cells, or islet cells, of the pancreas. Tumors that form in islet cells are called islet cell tumors, pancreatic endocrine tumors, or pancreatic neuroendocrine tumors, also called pancreatic NETs or PNETs. When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Most pancreatic NETs are functional tumors, which cause extra quantities of hormones, such as gastrin, insulin, and glucagon, that cause signs and symptoms.

Pheochromocytoma Page 1

This rare tumor starts in the chromaffin cells of the adrenal gland. These specialized cells release the hormone adrenaline during times of stress. Pheochromocytoma usually occurs in the adrenal medulla, the area inside the adrenal glands above the kidneys. These tumors increase the production of adrenaline and noradrenaline, hormones that increase blood pressure and heart rate.

Merkel cell cancer Page 1

Merkel cell cancer is highly aggressive, fast-growing, and rare. Merkel cell cancer may also be called neuroendocrine carcinoma of the skin or trabecular cancer.


Neuroendocrine Tumor Stages

After being diagnosed with a neuroendocrine tumor, the next important step is to determine the stage of the tumor. Effective treatment plans require accurate staging. Surgery may cure early-stage neuroendocrine cancer, but stage four cancer is incurable.

Generally, staging refers to the tumor’s location, if and where it has spread, and whether other body parts are affected. The higher the stage, the more advanced the cancer. There may be slight differences in staging based on the tumor's location.

  • Stage 1 — Cancer is present but has not spread beyond the origin site. This is also called early-stage cancer.
  • Stage 2 — Cancer is present and may have grown in size. It has not spread to other parts of the lymph nodes or other parts of the body.
  • Stage 3 — Cancer may have spread to nearby lymph nodes but has not spread to distant parts of the body.
  • Stage 4 — Cancer has spread to distant parts of the body. This is called advanced or metastatic cancer.

Stage 4 cancer is not always terminal but usually cannot be cured. Patients may need more aggressive treatment options to prolong their life and improve their quality of life.


Neuroendocrine Tumor Grades

In addition to the stage, doctors will assign a grade to your tumor. Grading describes how fast the tumor cells grow. NETs are divided into three grades:

  • Grade 1 (low grade) — slow growth.
  • Grade 2 (intermediate grade) — intermediate growth.
  • Grade 3 (high grade) — fast growth.

NETs are also defined by differentiation or the degree to which tumors resemble their cell of origin.

  • Well-differentiated NETs look more like healthy cells than cancerous cells.
  • Poorly-differentiated NETs look less like healthy cells and more like cancerous cells. Poorly-differentiated cells can be divided even further:
    • Small cell
    • Large cell

Diagnosis

NETs can be challenging to diagnose because they often grow slowly and only cause symptoms once they are advanced. However, some NETs can produce hormones that cause various symptoms, such as flushing, diarrhea, and wheezing.

The following tests may be used to diagnose NETs:

  • Blood and urine tests: These tests can look for elevated levels of hormones or other substances produced by NETs.
  • Imaging tests: Imaging tests such as CT, MRI, and PET scans can be used to locate NETs and determine their size and spread.
  • Biopsy: A biopsy is the removal of a small sample of tissue for examination under a microscope. This is the only definitive way to diagnose a NET.

Treatment

The treatment for NETs depends on several factors, including the type and location of the tumor, its grade (how aggressive it is), and whether it has spread.

The following treatment options may be used for NETs:

  • Surgery: Surgery is the primary treatment for localized NETs.
  • Somatostatin analogues (SSAs): SSAs are medications that bind to somatostatin receptors on NET cells and can help to slow tumor growth and relieve symptoms.
  • Targeted therapy drugs: Targeted therapy drugs work by targeting specific molecules involved in NET cells' growth and survival.
  • Radiation therapy: Radiation therapy can be used to kill NET cells and shrink tumors.
  • Chemotherapy: Chemotherapy is a medication that kills cancer cells throughout the body.

The Role of Theragnostics

Diagnosing and treating neuroendocrine tumors can be a complex process. However, the Sylvester team has expertise in an innovative approach known as theragnostics, which significantly refines this process.

Theragnostics utilizes radiolabeled compounds to locate NET cells through PET/CT imaging, enabling the tailoring of personalized treatment plans based on each patient's unique tumor characteristics. This approach combines diagnostic and therapeutic techniques to accurately and effectively locate and treat cancer cells.


Clinical Trials and Research

Sylvester is a hub for groundbreaking research and actively participates in clinical trials.

Recent trials have explored the synergy of combining established therapies with emerging treatments, aiming to amplify their effectiveness while minimizing side effects. Innovations in drug formulations and delivery methods are also under investigation, seeking to optimize therapeutic agents' bioavailability and targeting capability.

The department is also exploring the potential of peptide receptor radionuclide therapy (PRRT) and other targeted treatments in medical therapy. These innovations hold the promise of delivering highly specific treatment to tumor cells, minimizing systemic exposure and associated side effects.

Here are some key points about Sylvester's role in clinical trials:

  • Cutting-Edge Protocols: Sylvester is often among the first to employ new treatment methods under investigation, offering patients potential alternatives to traditional therapies.
  • Multi-Centric Collaboration: The center works in partnership with national and international research organizations, enabling a collaborative approach to medical breakthroughs.
  • Patient-Centric Approach: Clinical trials at Sylvester are designed with the patient’s safety and well-being in mind. Rigorous ethical standards are upheld, and patients are closely monitored for any adverse effects.
  • Translational Research: Sylvester's research often bridges the gap between laboratory discoveries and patient care, turning scientific insights into actionable treatments.
  • Access to a Range of Trials: The center offers Phase I to Phase IV clinical trials, exploring everything from early-stage investigational treatments to post-market surveillance studies.
  • Individualized Trial Matching: After a thorough assessment, patients may be matched with a clinical trial that specifically aligns with the characteristics of their NET, thereby maximizing the potential for effective treatment.

Through its dedicated clinical trials program, Sylvester is not just treating NETs based on existing medical knowledge, but actively contributing to the expansion of that knowledge. This allows Sylvester to remain at the forefront of innovations in NET management, offering hope and novel treatment options to its patients.


Neuroendocrine Tumor FAQ

Neuroendocrine tumors (NETs) are rare, with just 12,000 cases diagnosed each year in the United States. They can also mimic other conditions, making diagnosing NETs even more difficult.

If you or a loved one has recently been diagnosed with a neuroendocrine tumor, you probably have a lot of questions. Here are some common questions patients have about neuroendocrine tumors.

What is a Neuroendocrine Tumor? Page 1

A neuroendocrine tumor is a cancer that starts in the neuroendocrine cells. These specialized cells are nerve cells that produce hormones like endocrine cells. NETs can occur anywhere in the body.

Who Gets Neuroendocrine Tumors? Page 1

Anyone can develop a NET, but they are more common in some people than others:

  • Adults are more likely to develop NETs than children.
  • NETs are slightly more common in women than men, although it’s not clear why.
  • NETs are more common in white people than Black people, although the location of a NET varies by race.

Certain medical conditions can also increase the risk of developing a NET. These include:

  • Multiple endocrine neoplasia, type 1 (MEN 1) and type 2 (MEN 2) — A group of disorders that affect the endocrine system. The different types affect different genes and hormones, and have different signs and symptoms.
  • Neurofibromatosis — A group of genetic disorders that cause tumors to form on nerve tissue.
  • Tuberous sclerosis — A genetic disorder caused by gene changes that cause non-cancerous tumors to grow in the brain and other parts of the body.
  • Von Hippel-Lindau disease — An inherited, multi-system disorder that causes non-cancerous tumors to grow in certain parts of the body.
How Does a Neuroendocrine Tumor Form? Page 1

Experts do not know what causes NETs to form. However, patients with multiple endocrine neoplasia with changes in the MEN1 gene have a higher chance of developing carcinoid tumors. The MEN1 gene may play a role in the development of NETs in the lungs and digestive tract.

What are the Signs and Symptoms of a Neuroendocrine Tumor? Page 1

The signs and symptoms of a NET depend on where the tumor is located, and whether it is functional or non-functional. Functional NETs produce excess hormones and cause symptoms, while non-functional NETs do not produce hormones or produce very little hormones.

Common neuroendocrine tumor symptoms include:

  • Fatigue and weakness
  • Nausea or vomiting
  • Pain from a growing tumor
  • Unexplained weight loss

Other symptoms that can occur with functional NETs include:

  • Diarrhea
  • Dizziness or shakiness
  • Frequent urination
  • Increased thirst
  • Skin rash or flushing
How is a Neuroendocrine Tumor Diagnosed? Page 1

Diagnosing a neuroendocrine tumor depends on where it is located in your body. Generally, diagnostic tests include:

  • Physical exam to look for swollen lymph nodes or other signs of excess hormone production.
  • Imaging tests such as CT, MRI, or ultrasound to take pictures of the tumor.
  • Biopsy to remove a sample of the tumor cells.
  • Blood or urine test to look for signs of excess hormone production.
What are the Treatment Options for Neuroendocrine Tumors? Page 1

Treatment options for neuroendocrine tumors depend on the type of tumor, its location, and whether you are experiencing symptoms.

Treatment options include:

  • Surgery. Surgery removes the tumor as well as some of the healthy surrounding tissue. If the surgeon cannot remove the tumor completely, they will remove as much of it as possible. Surgery is the best option for treating carcinoid tumors.
  • Chemotherapy. Chemotherapy uses strong drugs to kill the tumor cells. Chemotherapy is usually recommended for advanced tumors that cannot be removed with surgery. It can be used alone or in combination with other treatments.
  • Targeted drug therapy. Targeted drug therapy uses drugs that target abnormalities within cancer tumor cells. Targeted drug therapy is often combined with chemotherapy for advanced tumors.
  • Peptide receptor radionuclide therapy. Peptide receptor radionuclide therapy uses high doses of radiation to destroy or slow the growth of tumors and reduce the side effects of the disease.
  • Radiation therapy. Radiation therapy uses high-energy X-rays to destroy tumor cells. External-beam radiation therapy is the most common type of radiation treatment for neuroendocrine tumors.
  • Medication. Medication is used to destroy cancer cells. A patient may take medication intravenously (through the vein using a needle) or in a pill or capsule form. They may also receive an injection into the muscle.
Are Neuroendocrine Tumors Hereditary? Page 1

NETs do not appear to be hereditary, meaning they don’t run in families. However, some people may have a genetic risk. People who have multiple endocrine neoplasia have a higher risk of getting carcinoid tumors.

What is the Prognosis for a Neuroendocrine Tumor? Page 1

A prognosis is a doctor’s best estimate of how a disease will affect you and how it will respond to treatment. The prognosis for a NET depends on several different factors. NETs caught in the early stages before they have had a chance to spread to other parts of the body are easier to treat and have favorable prognoses. NETs that are caught in the later stages and have spread have a less favorable prognosis.

Other factors that can affect the prognosis and survival rate for a neuroendocrine tumor include:

  • Grade and differentiation
  • Hormone production (functional vs. non-functional)
  • Location
  • Size

Why Choose Sylvester Comprehensive Cancer Center?

NCI-Designation: Sylvester's recognition by the National Cancer Institute underscores its excellence in cancer care and research, providing an edge in NETs management.

Dedicated NETs Program: Spearheaded by Dr. Aman Chauhan, the program focuses on advancing NETs treatment and research, catering to the specific needs of NETs patients.

Clinical Trials Innovation: With ongoing trials exploring novel treatments, Sylvester is a hub for pioneering NETs research, offering patients access to groundbreaking therapies.

High-volume experience with stomach, gastric, and neuroendocrine cancers. We have patients in 22 states with stomach, gastric, and neuroendocrine cancers, so we have the expertise to provide better outcomes with fewer potential complications.

Theragnostic Expertise: Sylvester's approach integrates diagnosis and treatment, utilizing theragnostics to tailor precise treatment plans for NETs, enhancing therapeutic effectiveness.

Advanced Diagnostics: Employing modern diagnostic tools such as PET/CT scans and hormone level assessments, Sylvester ensures accurate identification and staging of NETs, forming the bedrock for effective treatment strategies.

Multidisciplinary Expertise: A team of seasoned professionals collaborates to deliver a comprehensive, personalized treatment plan, ensuring optimal care for NETs patients.

Minimally invasive NanoKnife® radiosurgery. We provide more treatment options for patients with inoperable or difficult-to-reach tumors, producing little pain and requiring only a brief hospital stay.

Advanced radiation therapy options, like proton therapy, ViewRay (one of just five centers nationwide), and RapidArc®, offer intensity-modulated external radiation therapy (IMRT). Our leading-edge technology provides shorter treatment times, pinpoint accuracy in tumor targeting, and less damage to surrounding healthy tissue.

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