Neuroendocrine Tumors | MedStar Health

What are neuroendocrine tumors?

The brain and the nervous system provide the signals to the endocrine system to produce hormones that regulate bodily functions. Since these two systems are so interdependent, they are often referred to as the neuroendocrine system. Neuroendocrine tumors (NETs) are a broad category of abnormal growths that begin in neuroendocrine cells, which are similar to nerve cells and hormone-producing cells. These tumors often produce abnormal amounts of hormones, affecting how the nervous system works.

All neuroendocrine tumors are malignant (cancerous), although they grow at varying rates. They are rare and can occur in any organ in the body. However, they are most often found in the lungs, appendix, small intestine, rectum, and pancreas. Doctors diagnose and treat these tumors based on the types of cells involved and their location in the body.

Symptoms and risk factors

What are the signs of a neuroendocrine tumor?

Not every neuroendocrine tumor will cause symptoms. Your symptoms will vary based on the location of your tumor and its impact on hormone production. Each variation of neuroendocrine tumor presents specific symptoms. These may include:

  • Hyper or hypoglycemia
  • Unusual weight changes
  • Unusual anxiety
  • Unexplained lumps
  • Jaundice
  • Unexplained bleeding
  • Unusual bowel or bladder changes
  • Ongoing night sweats

Who is at risk for developing a NET?

People may have a higher risk for developing these tumors because of certain factors, such as:

  • Gender: men are more likely than women to develop pheochromocytoma
  • Age: Patients with pheochromocytoma are generally between 40-60 years old
  • Genetics: Certain inherited genetic syndromes, such as Multiple endocrine neoplasia, type one and two (MEN1 and MEN2), or Von Hippel-Lindau disease, may be more likely to develop cancer

Screening and prevention

Can neuroendocrine tumors be prevented?

We don't know the exact cause of neuroendocrine tumors. Therefore, you can't prevent this type of cancer.

Diagnosis

How are neuroendocrine tumors diagnosed?

First, your doctor will perform a physical exam and ask questions about your symptoms, medical history, and family medical history. Then, your doctor may recommend one or more tests to detect the presence of a tumor:

  • Biopsy: This is the most definitive diagnostic method. Doctors examine tissue samples for evidence of a tumor. Tissue samples are collected in different ways, depending on the location of the tumor.
  • Blood and urine tests: The levels of hormones produced under certain circumstances may be present in blood and urine and indicate a possible tumor.
  • Imaging: With a computed tomography (CT) scan, MRI, or X-ray, the doctor can usually verify the existence of a neuroendocrine tumor, as well as determine its exact size and placement.

Types of neuroendocrine tumors

Neuroendocrine tumors are often classified by where they begin in the body.

At MedStar Health, our endocrine specialists treat several types of neuroendocrine tumors, including:

  • Insulinoma: This rare and often benign pancreatic tumor derives from beta cells and secretes insulin.
  • VIPoma (Verner Morrison syndrome): This rare endocrine tumor originates from non-β islet cells of the pancreas, which produces vasoactive intestinal peptide (VIP).
  • Pheochromocytoma: This tumor originates in the adrenal gland.
  • Carcinoid tumors: These tumors most commonly develop in the lung, intestinal tract, or thymus.
  • Medullary thyroid carcinoma: This form of thyroid cancer comes from the C-cells of the thyroid, which produce a hormone called calcitonin.
  • Adrenal tumors: Rarely, tumors can develop in the adrenal glands, which are located on top of the kidneys. Most are non-cancerous and develop in the outer layer of the adrenal glands called the cortex.
  • Paraganglioma: A rare adrenaline-producing tumor outside the adrenal gland that may develop at various body sites, including the head, neck, thorax, and abdomen.
  • Islet cell tumors (islet cell cancer): This pancreatic neuroendocrine tumor arises in the pancreas.

Treatments

How is a NET treated?

Our specialists take a multidisciplinary approach to care, working together to provide comprehensive cancer treatment involving oncologists, gastroenterologists, surgeons, and other healthcare specialists. Your treatment of a neuroendocrine tumor depends on several factors, including:

  • The type of tumor
  • The tumor's location in the body
  • Whether it produces excess hormones
  • How aggressive it is
  • Whether it has spread to other parts of the body.

Treatment is individualized to each patient, and your cancer team will help you understand all of your options so you can make an informed decision about your care.

Surgery

Surgery is one of the primary treatments for neuroendocrine tumors. During surgery, a surgeon will try to remove all of the tumor—or as much of it as possible. The type of surgery you undergo will vary based on where the tumor is located and whether or not it has spread. Even if patients with a metastatic NET may benefit from surgery, which can help to alleviate symptoms or side effects of the cancer.

Chemotherapy

Chemotherapy is the use of medication taken by mouth or via injection to kill cancer cells throughout the body. Depending on the type of NET, chemotherapy may play a role in treatment. This treatment is guided by a medical oncologist who will work closely with the other members of your care team to determine if you would benefit from chemotherapy alone or in combination with other treatments.

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Immunotherapy and targeted therapy

Immunotherapy harnesses the body's natural immune system to fight back against cancer, although it's more often used to treat other cancer types. Targeted therapies use medications to identify and target certain proteins or genes, helping to slow or stop the growth of cancerous cells. Depending on your tumor, your doctors may do additional testing to predict whether or not your tumor would respond to certain targeted treatments, including those under investigation in clinical trials.

Learn More About Immunotherapy

Learn More About Targeted Therapy

Radiation therapy

Radiation therapy uses high-energy x-rays or particles to kill cancerous cells. Most commonly, this is delivered externally using technology that delivers radiation from outside of the body. At MedStar Health, our radiation oncologists have access to all of the most advanced radiation techniques and machines, including stereotactic body radiation therapy delivered using Cyberknife and proton therapy. These highly precise forms of radiation may be used to treat tumors considered hard-to-reach with surgery or inoperable while limiting radiation exposure to healthy tissue. Radiation can also be targeted at symptomatic regions for palliation. If you are a candidate for radiation therapy, a radiation oncologist will develop an individualized plan for your specific radiation protocol based on your tumor type, location, stage, and your overall health.

In addition, certain NETs in the pancreas or gastrointestinal tract may benefit from a new, FDA-approved treatment, Lutathera. This form of targeted radiotherapy is more broadly known as peptide receptor radionuclide therapy (PRRT). It's administered intravenously, using radioactive drugs to destroy cancer cells by binding to cell receptors present in certain tumors.

Learn More About Radiation Therapy

Hormone therapy

If your NET produces excess hormones, your cancer team may recommend certain medications that can help regulate hormone production. For example, somatostatin is a hormone in the body that regulates how insulin and glucagon are released. Somatostatin analogs are drugs that can mimic this hormone, helping to control hormones released by a tumor. While they don't typically shrink tumors, they can help to manage symptoms and slow tumor growth of certain NETs.

Learn More About Hormonal Therapy

Looking for expert cancer care?

With multiple locations throughout the region, patients have access to many of the nation’s renowned cancer specialists offering high quality care, second opinions and a chance for better outcomes close to where they live and work. Georgetown Lombardi Comprehensive Cancer, one of the nation’s comprehensive cancer centers designated by the National Cancer Institute (NCI), serves as the research engine allowing patients access to clinical trials that often lead to breakthroughs in cancer care.

Our providers

Neurosurgeon examining x-ray scans of senior patient

Expert care for neuroendocrine tumors

Getting the care you need starts with seeing one of our specialists.

Our locations

Distance from Change locationEnter your location

MedStar Georgetown Cancer Institute at MedStar St. Mary’s Hospital

25500 Point Lookout Rd.
First Fl.
Leonardtown, MD 20650

MedStar Franklin Square Cancer Center at Loch Raven Campus

5601 Loch Raven Blvd.
Russell Morgan Building
First Floor
Baltimore, MD 21239

MedStar Georgetown Cancer Institute at MedStar Franklin Square Medical Center

9103 Franklin Square Dr.
The Harry and Jeanette Weinberg Cancer Institute
Suite 220
Baltimore, MD 21237

Why choose us

At MedStar Health, our multi-specialty team of neuroendocrine cancer experts work together to personalize care that will lead to your best outcomes. Patients come to us for cancer care because:

Treatment plans involve the collective input of experts in different fields. Your treatment options will be as unique as you are, taking into consideration your specific needs and tumor's characteristics. Depending on the location of your neuroendocrine tumor, your team may involve specialists in:

  • Gastroenterology
  • Endocrinology
  • Endocrine surgery
  • Surgical oncology
  • Transplant surgery
  • Medical oncology
  • Radiation oncology
  • Pathology
  • Radiology
  • Interventional radiology
  • Genetic counseling
  • Social workers
  • Dietitians
  • and more

These cancer specialists meet regularly to discuss complex cases and design personalized treatment that considers every aspect of your care, physically, emotionally, and more.

We offer all of the latest therapies available today. From robotic, laparoscopic, open, and hybrid surgical options to emerging medical and radiation therapies, our doctors are equipped to care for even the most complicated neuroendocrine tumors. Whenever possible, our goal is to remove the tumor and minimize the chance of spreading. We also work diligently to stabilize hormone levels to help you maintain your quality of life during and after treatment. Through our research engine, Georgetown Lombardi Comprehensive Cancer Center, you may be eligible for a clinical trial that offers a promising treatment option not yet widely available.

We care for all of you, not just your cancer. With a variety of comprehensive services designed to support, encourage, and guide you throughout your journey with cancer, we're here to treat all of you. As a MedStar Health patient, you'll have access to the entire breadth of our specialty care, which ensures we can address any need that arises. Our holistic approach involves nutritional support, pain management, psychosocial services, rehabilitation, and more so you can live the fulfilling life you deserve.

Awards and recognition

Recipient of an Accreditation with Commendation, the highest level of approval, from the American College of Surgeons’ Commission on Cancer (CoC)

Numerous surgeons, medical oncologists, and radiation oncologists recognized as "Top Doctors" by both Baltimore Magazine and the Washingtonian

Magnet® designation from the American Nurses Credentialing Center (ANCC)

Clinical trials and research

Clinical trials

Clinical trials for neuroendocrine tumors allow researchers to study emerging treatment options that may be better than the current standard of care. By participating in a cancer clinical trial, eligible patients may benefit from a breakthrough treatment before it becomes more widely available.

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Research

Our doctors and researchers continue investigating safer, more effective ways to diagnose and treat cancers affecting the endocrine system through our research engine, the Georgetown Lombardi Comprehensive Cancer Center—Washington, D.C.,'s only comprehensive cancer center designated by the National Cancer Institute.

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Support services

A neuroendocrine tumor diagnosis can be overwhelming, but we're here to support, encourage, and guide you through treatment and beyond. Here, you'll find a wide range of services, specialties, and programs to help you navigate your cancer journey physically, emotionally, and mentally.

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Genetic counseling

Because a small portion of neuroendocrine tumors are linked to inherited syndromes, our board-certified genetic counselor can help you understand the benefits, risks, and limitations of testing for specific gene mutations. Counseling can also help you interpret test results and determine appropriate next steps for managing your cancer risk.

Learn More About Genetic Counseling

Personalized rehabilitation

Cancer rehabilitation can help patients regain mobility and independence over different aspects of their lives. Depending on your needs, you may benefit from a wide range of services, from physical therapy, occupational therapy, nutritional planning, or counseling.

Learn More About Cancer Rehabilitation

Survivorship

Your care doesn't end after treatment. As part of your survivorship plan, your doctor will recommend routine visits to help check for recurrence through blood or imaging tests. In addition, your care team will help to manage any side effects related to your cancer treatment or condition to minimize its impact on your quality of life as you return to your daily activities.

Learn More About the Cancer Survivorship Program