Treatment for Neuroendocrine Tumors (NETs) at Virginia Oncology Associates
What are Neuroendocrine Tumors?
Neuroendocrine tumors, also called NETs, are rare and account for less than one percent of all malignant (cancerous) disorders in the United States.
Neuroendocrine tumors form in hormone-producing cells, called neuroendocrine cells. These cells develop anywhere in the body but most often grow in the digestive tract, lungs, pancreas, appendix, or rectum. They are typically small and develop slowly over many years.
Neuroendocrine tumors are not only rare but complex and can be difficult to diagnose. The cancer specialists at Virginia Oncology Associates have put together this information about detection, diagnosis, staging, and treatment options for neuroendocrine tumors.
Neuroendocrine Tumor Types
The type of neuroendocrine tumor is determined by where the tumors originated. These may be generally referred to as gastrointestinal (GI) NETs, pancreatic NETs, or lung NETs, which can then be broken down into subsets including:
- Carcinoid tumors
- Small cell carcinoma
- Large cell carcinoid tumor
- Medullary thyroid carcinoma
- Paraganglioma
- Pheochromocytoma
- Adrenal cancer
- Merkel cell carcinoma (neuroendocrine carcinoma of the skin)
The type of neuroendocrine tumor you have, along with other factors, will influence the treatment plan your oncologist will recommend for you. Other factors include the location of the tumor, whether it produces excess hormones, how aggressive it is, and whether it has spread to other parts of the body.
Signs and Symptoms of Neuroendocrine Tumors
Similar to other cancer types, the signs of NETs are not obvious in the beginning. If symptoms are experienced, they differ based on the location of the tumor. Often these symptoms are the cause of other conditions. Having more than one symptom does not mean neuroendocrine tumors have developed, but you should be aware of any potential signs in case they persist, and a doctor’s analysis is needed.
The most common symptoms for NETs can include:
- Pain in the abdomen
- Intense skin flushing
- Unexplained weight loss
- Loss of appetite
- Heart palpitations
- Diarrhea
- Wheezing
- Heartburn
- Fatigue
Detecting and Diagnosing NETs
The rarity of neuroendocrine tumors can make them difficult to detect and diagnose. Often, NETs are found unexpectedly during x-rays or medical procedures completely unrelated to the tumor.
If a neuroendocrine tumor is suspected, a doctor will perform a complete physical examination that includes a review of your medical and family history. From there, testing is required to ensure an accurate diagnosis is made. The tests performed will depend on the type of tumor, its location, its production of excess hormones, how aggressive it is, and whether it has spread to other parts of the body.
The diagnostic tests we may use to diagnose NETs include:
- Blood and/or urine tests
- Molecular testing of the tumor
- Endoscopic ultrasound
- Biopsy
- X-ray
- MRI
- CT scan
- PET or PET-CT scan (Gallium 68)
- Nuclear imaging (OctreoScan™)
Staging of Neuroendocrine Tumors
If you have been diagnosed with one or more NETs, the next step is to determine the stage of the cancer which means finding out the extent or spread of the tumor. The stage of the NET will help your oncologist determine the best course of treatment for you.
Some neuroendocrine tumors, like gastrointestinal (GI) tract NETs and pancreatic NETs, have their own staging systems. Other NETs use the staging system of other cancers. For example, the staging of a lung NET is the same as the staging of non-small cell lung cancer. Most neuroendocrine tumors are classified as low-grade tumors because of how slowly they grow, but this is not always the case.
Staging can seem confusing, especially since it is based on where the tumors originally developed. We urge you to speak with the cancer care team at Virginia Oncology Associates if you have any questions or concerns.
NET Treatment Options
The details of your NET diagnosis play a huge role in the type of treatment plan that may work best for you. The location of the tumor, the grade, the growth rate, as well as the severity of symptoms are all contributing factors to making a prognosis and determining a treatment plan. When diagnosed and treated appropriately, most patients with neuroendocrine tumors can enjoy a quality life for many years.
Watchful Waiting
If detected early, watchful waiting, also known as active surveillance, may be the recommended treatment for NETs. It may also be used for patients who are older or who have underlying health conditions. During this period, an oncologist will monitor and test to track the progression of cancer. If they feel the NETs are slow-growing, they may recommend waiting to receive treatments that could really impact your quality of life. While active surveillance does not take immediate steps to reduce or eliminate cancer, your medical team can closely identify when medical treatment options should begin.
Surgery to Remove NETs
If surgery is possible, oncologists often prefer it as a first treatment option. The location and size of the tumor will likely determine if surgical removal is possible. However, even if the tumor cannot be removed completely, partial removal may be a solution. Doing so can help relieve symptoms, prevent problems, and even slow the tumor’s progression. Surgery provides relief from symptoms and delays the need for systemic treatments.
Liver-Directed Therapy
Unfortunately, 90% of NET patients will have cancer spread to the liver. Embolization is a procedure that uses tiny particles to block blood vessels. Without a blood source, the tumor is unable to grow.
Advanced techniques of this treatment include:
- Bland embolization: Uses oil, gelatin, or other small particles to cut off the artery’s supply.
- Chemoembolization: Combines chemotherapeutic medications with embolization. This causes the medication to get trapped in the tumor.
- Radioembolization: Uses a concentrated combination of radiation targeting the tumor.
Hormonal Therapy–Somatostatin Analogs (SSAS)
Biotherapy with SSAS is a standard option for treating symptoms of NETs. It has also been proven to slow down tumor growth. Biotherapy is a type of treatment that uses materials from living organisms to treat the disease. There are two types of SSAS available:
- Octreotide: This medication is available in short-acting and long-acting doses. Short-acting medications may provide immediate relief lasting 4-6 hours and must be given 2-4 times a day. This type of treatment may be more appropriate for flare-up symptoms.
- Lanreotide: This medication is available in long-acting doses. Long-acting medications are best for managing chronic symptoms that are more regular and persistent.
Chemotherapy
Chemotherapy is a NET cancer treatment given to patients with more aggressive (fast-growing) tumor growth. The oncologist may refer to them as neuroendocrine carcinomas. This type of cancer often returns but may respond better to chemotherapy. Chemotherapy is especially a preferred treatment method for patients with NETs that begin in the pancreas. Chemotherapy is given either orally or through an IV. Chemotherapy may be suggested under the following conditions:
- The tumor is likely to return.
- The tumor has spread to other organs.
- The tumor cannot be surgically removed.
- There are severe symptoms associated with NETs.
- Other medications, such as somatostatin or targeted therapies proved unsuccessful.
Targeted Therapy
Targeted therapies focus on blocking specific genes or proteins in a tumor. Eliminating those proteins or genes makes it so the cancer cells are unable to grow or spread. This type of cancer treatment uses medications and other substances to target molecules in the cancer cells. Targeted therapies may be especially beneficial for cancer cells that are slower growing.
Keep in mind that not all types of tumors have the same targets. Your oncologists will need to identify your tumor’s targets before choosing a course of action. The doctor will run a series of tests to determine the nature of the tumor.
Several medications fall into these categories; however, the most recent option available at Virginia Oncology Associates is Lutathera®.
Lutathera® is known as radioisotope therapy. It is a treatment for cancers affecting the neuroendocrine cells of the pancreas and parts of the stomach. This prescription medication has been proven to slow or stop the growth of the tumor. It works as a targeted radioactive therapy that delivers the medication directly to the tumor while sparing healthy cells.
Biological Therapy
Biological therapy or immunotherapy is one possible NET treatment that may be recommended. Your oncologist may use interferons which are materials produced naturally in your body or in a research lab. These materials are used to strengthen your immune system.
Once it is strong enough, your immune system can fight cancer cells and reduce symptoms such as diarrhea and flushing. Interferons may also shrink tumors.
Radiation Therapy
External beam radiation therapy is used when treating pain or pressure associated with your cancer. This treatment works especially well for patients with pancreatic NETs that have spread to the bone. Radiation therapy can also be useful for treating tumors that are located deeper inside the body. Radiation therapy is usually given to patients daily, on a set schedule, over a period of several weeks. Chemotherapy targets cancer by placing medication inside the body; however, radiation therapy uses a machine that targets cancer from the outside.
Side effects of radiation therapy are often mild. These symptoms may include fatigue, nausea, and redness of the treated area. Most patients say the side effects decrease after their treatment is completed.
Clinical Trials
Clinical trials can be an excellent option for NET patients. Clinical trials pair candidates with new and emerging treatment options. You may be interested in clinical trials if other treatments are not providing relief. Speak with an oncologist if you would like to learn more about clinical trials available to NET patients at our clinics throughout Hampton Roads-Tidewater.
Find a Neuroendocrine Specialist
For the neuroendocrine specialists at Virginia Oncology Associates, there is no one-size-fits-all approach, as every case and every patient are different. Request an appointment with a cancer specialist at any of our locations in Hampton Roads-Tidewater, Virginia, or northeastern North Carolina. Our cancer care team can answer questions specific to your individual diagnosis, as well as provide a second opinion on treatment for neuroendocrine tumors.
