Rare Disease Day: Neuroendocrine Tumors
Did you know Feb. 28 is Rare Disease Day? The zebra is the official symbol of rare disease in the United States because each stripe is unique, much like rare diseases! While there are over 7,000 rare diseases, there are commonalities that unite the rare disease community.
There is a well-known and overused saying in medicine that, “If you hear hoofbeats, think of a horse, not a zebra,” since most illnesses will fall into one of a handful of “common conditions.” Patients with uncommon medical conditions, like Neuroendocrine Tumor (NET), are marginalized within the healthcare system due to the rarity of their disease and the lack of readily available medical providers that are familiar with the diagnosis and treatment of this cancer.
Neuroendocrine cancers are the “zebras,” making up just 2% of all cancers diagnosed each year in the United States. As such, the zebra has become the symbol for NET.
The most important aspect of this day is to raise awareness around rare diseases. Today, we’d like to raise more awareness around neuroendocrine tumors, what they are and common questions our physicians receive.
What is a neuroendocrine tumor?
A neuroendocrine tumor (NET) is a rare hormone-producing tumor that can originate anywhere in the body. While some NETs are benign, carcinoid tumors are the most commonly detected NETs, and they are usually found in the lungs or the gastrointestinal tract. Neuroendocrine tumors are cancers that begin in specialized cells called neuro cells. These have very similar traits to nerve cells and hormone-producing cells, making them very hard to detect.
Chemoembolization and radiofrequency ablation are two treatments that patients with NETs may undergo. When and why is chemoembolization used instead of radiofrequency ablation for carcinoid tumor metastases in the liver?
“Radiofrequency ablation is conducted one at a time. This happens by taking a needle-like probe into a tumor, and whether it’s radiofrequency ablation or not, we use a microwave ablation,” says Dr. Philip Boudreaux. “Both are similar but use a different radiofrequency to deliver energy. This process basically cooks the tumor, like you cook an egg in the microwave while being mindful of protecting blood vessels and other structures in the liver.”
The ablation doesn’t work well for tumors larger than 3 centimeters. If the tumor is any larger, microwave ablation may not be the best technique. The liver-directed therapy is focused on multiple smaller tumors. We often do these in sequences if there are several small tumors. If the tumor is too large, liver-directed therapy may not be the best option. Instead, surgery may be required to remove the tumor.
If less than fifty percent of the liver is occupied by the tumors and surgery is not an option, chemoembolization is often the best option. If more than fifty percent of the liver is occupied with a tumor, you may have to treat it in small sections at a time. Each patient receives an individualized plan based on the tumor, history, rate of disease progression and other factors.
After Peptide Receptor Radionuclide Therapy (PRRT), some patients experience low white or red blood cell counts. A follow-up series may not be recommended because of this. Would a blood or bone marrow transfusion be possible or recommended if the count drops too low?
This is one of the rare side effects of this type of therapy. This is a radioactive drug that binds to the tumor, like a guided missile that “cooks” it off. Sometimes the radiation from PRRT affects the bone marrow. The bone may have a hard time making enough white or red blood cell counts or platelets. Sometimes it can be all the above. In most cases, if you wait long enough, the counts will recover. Rarely the effect is permanent. If the counts go low enough, patients may need a transfusion to prevent anemia or platelet transfusions to minimize spontaneous bleeding. Unfortunately, white blood cells hurt the host more than they help, so we do not give white blood cell transfusions. We sometimes use medications that can help stimulate the growth of bone marrow to help recover the cells.
What is the best scan to detect carcinoid tumors?
“The best scan to detect tumors is a Gallium-68 PET Scan or a Copper-64 PET Scan. Copper-64 is a newer scan. The Copper isotope is easier to handle and deals with less radiation. I predict it will replace the Gallium-68 PET Scan soon,” says Dr. Philip Boudreaux. The Copper-64 PET Scan is Food and Drug Administration approved and readily available. To follow and assess disease progression in the liver, we have found that the MRI is much better to evaluate the liver than a CT scan.
Why should a patient seek care with Ochsner’s NET program?
Our program is an affiliation between Ochsner Medical Center – Kenner and Louisiana State University Health Science Center in New Orleans. Our doctors and staff specialize in the diagnosis and management of all forms of NETs. They have treated thousands of patients and have proven outcomes.
We are the largest multispecialty team in the country dedicated to treating NETs, and we take a multidisciplinary approach to your care. With more than 50 years of combined experience, our team works with referring physicians on diagnosis and disease management decisions. Because of this, people from all over the country have chosen to come to us for their care. We are following over 4,000 active patients and have performed over 2,000 operations for NETS.