Neuroendocrine tumors: what to know about these rare cancers

When it comes to cancer, you’re probably familiar with the more common cancers in Louisiana and the U.S.: breast cancer, lung cancer, colorectal cancer and prostate cancer, but you may not know much about rare cancers. Neuroendocrine tumors are a group of cancers that impact neuroendocrine cells in different parts of the body. Each year, approximately 12,000 new cases of neuroendocrine-related cancers are diagnosed in the U.S.

People with rare cancers such as neuroendocrine tumors require coordinated and advanced treatment. They can find that care at East Jefferson General Hospital through the Rare Cancer program.

Rare cancer in the U.S.

According to the American Society of Clinical Oncology, more than 171,000 Americans are now living with some type of neuroendocrine tumor. Diagnosis of these rare cancers has been increasing over the past few decades, in part due to increased awareness about them and improved diagnostic tools.

As a result, neuroendocrine tumors are now the focus of more research and advanced clinical trials, bringing hope and potential for the future.

What neuroendocrine tumors are

While the word contains neuro, these rare cancers are not related to the neurological system or the brain. Instead, these tumors occur in neuroendocrine cells, which are found throughout the body. Neuroendocrine cells are responsible for sending and receiving messages in the body to help with essential functions, such as reproduction and metabolism.

While neuroendocrine tumors can form anywhere there are neuroendocrine cells, such as in the pancreas, they most commonly develop in the GI tract and the lungs. In some cases, such as with neuroendocrine tumors in the lungs, these cancers can be subtypes of more common cancers.

How these rare cancers are diagnosed and treated

There are no routine screening tests for rare cancers like neuroendocrine tumors. Most cases of these rare cancers are discovered after a person experiences symptoms or while receiving medical attention for another health issue.

Symptoms of neuroendocrine tumors vary by the part of the body affected. Someone with a neuroendocrine tumor in the lungs may experience difficulty breathing, wheezing and chest pain, while someone with a GI neuroendocrine tumor may experience digestive issues.

A neuroendocrine tumor would be diagnosed with a combination of tests, including blood or urine tests to check hormone levels, imaging scans, and biopsies. If a tumor is found, it will be further classified as functional or non-functional.

Functional neuroendocrine tumors make and release hormones, which cause symptoms. The majority of neuroendocrine tumors are considered nonfunctional, which means they don’t release enough hormones to cause noticeable symptoms in an early stage.

Treatment of these rare cancers will depend on factors, including where the tumor is located, whether it’s functional or nonfunctional, the stage of the cancer and how quickly it’s growing. A team of providers, including oncologists and specialists in fields such as endocrinology and gastroenterology, will collaborate to determine an optimal treatment plan.

Treatment for neuroendocrine tumors may include many different therapies, including active surveillance, chemotherapy, immunotherapy, radiation therapy, surgery, targeted therapy or a combination of therapies. In some cases, a clinical trial may be recommended based on the patient’s health status and whether there is ongoing research.

Outcomes for these rare cancers vary, but many people diagnosed with neuroendocrine tumors can live long, full lives after treatment. The overall five-year relative survival rate for those diagnosed with a GI tract neuroendocrine tumor is 94%, for example, while the five-year relative survival rate for those diagnosed with a lung neuroendocrine tumor is 89%.

As research continues and innovative treatment options emerge, rare cancers are expected to become even more treatable.

When you’re diagnosed with a rare cancer, you may be uncertain where to turn for care. Have peace of mind in knowing the East Jefferson General Hospital Rare Cancer program is here to help.