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. Tumors that affect the functioning of cells within this system are collectively called neuroendocrine tumors.

The primary types of neuroendocrine tumors are:

  • Pheochromocytoma, which affects production of adrenaline and often presents in the adrenal glands
  • Neuroendocrine tumors, which is a generic term for tumors that affect hormones in major organs (such as the pancreas)

What are neuroendocrine tumors?

These are abnormal growths that begin in neuroendocrine cells, which are similar to nerve cells and to hormone-producing cells. These tumors grow in the organs of the neuroendocrine system, causing them to produce abnormal amounts of hormones, which then affect how the nervous system works.

Neuroendocrine tumors can be non-cancerous or cancerous. They are rare and can occur anywhere in the body, but most often are found in the lungs, appendix, small intestine, rectum, and pancreas.

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

  • Insulinoma: A tumor of the pancreas that is derived from beta cells and secretes insulin; rare and most often benign.

  • VIPoma (Verner Morrison syndrome): A rare endocrine tumor  originating from non-β islet cells of the pancreas, which produces vasoactive intestinal peptide (VIP).

  • Pheochromocytoma: A tumor of the adrenal gland.

  • Carcinoid tumors: Tumors of the lung, intestinal tract, or thymus.

  • Medullary thyroid carcinoma: A form of thyroid cancer that comes from the C-cells of the thyroid, which produce a hormone called calcitonin.

  • Adrenal cancer: A rare form of cancer that develops 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 cancer: A pancreatic neuroendocrine tumor is a neuroendocrine tumor that arises in the pancreas.

Risk factors

People are at a higher risk for developing neuroendocrine tumors because of certain factors. These may include:

  • Gender: Men are more likely than women to develop pheochromocytoma
  • Age: Pheochromocytoma patients are generally between the ages of 40 and 60
  • Genetics

Symptoms

Each variation of neuroendocrine tumor presents specific symptoms.

  • Pheochromocytoma
    • Elevated blood pressure
    • Damp and sticky skin
    • Unusual anxiety
    • Heart palpitations
    • Nausea, headaches, fever
  • Neuroendocrine Carcinoma
    • Hyper or hypoglycemia
    • Unusual weight changes
    • Unusual anxiety
    • Unexplained lumps
    • Jaundice
    • Unexplained bleeding
    • Unusual bowel or bladder changes
    • Ongoing night sweats

Diagnosis

Doctors can detect the presence of neuroendocrine tumors in the following ways:

  1. Biopsy: This is the most definitive diagnostic method. Doctors examine tissue samples for evidence of a tumor.
  2. Blood and urine tests: The levels of hormones produced under certain circumstances are present in blood and urine and are indicative of possible tumors.
  3. Imaging: With a CT or MRI scan or an X-ray, the doctor can usually verify the existence of a neuroendocrine tumor, as well as determine its exact size and placement.

A diagnosis of neuroendocrine tumor

After examining the results of one or more of these tests, your doctor may inform you that you have a neuroendocrine tumor. Treatment is based on where the tumor is located, how big it is, whether it is metastasizing and the patient's general health.

Treatments

Treatment of a neuroendocrine tumor depends on the type of tumor, its location, whether it produces excess hormones, how aggressive it is, and whether it has spread to other parts of the body.

Our specialists take a multidisciplinary approach to care and work together with other medical specialists to provide comprehensive treatment including oncologists, gastroenterologists, and surgeons.

Treatment options for neuroendocrine tumors often include:

  • Surgery to remove all or most of the tumor
  • Chemotherapy, in cases where tumors have spread to the liver
  • Medication to help stop the tumor from growing and to regulate hormone production
  • Removing a portion of the liver, in cases where tumors have spread to the liver

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.