About Neuroendocrine Carcinoid Tumors

Overview
Neuroendocrine carcinoid tumors are a rare type of cancer that typically develop in the digestive (GI) tract, lungs or pancreas. Less commonly, they may develop in the thyroid gland, parathyroid glands, pituitary gland, adrenal glands, or thymus. They are usually slow-growing. Neuroendocrine cells, which act like nerve cells and hormone-making cells, are responsible for the development of these tumors. Some neuroendocrine carcinoid tumors make hormones, which cause symptoms. More commonly, neuroendocrine carcinoid tumors do not make hormones (or very little hormone). People with tumors that don’t make hormones may not initially have symptoms until the tumor impacts an organ.
Causes and Risk Factors

Neuroendocrine carcinoid tumors develop from neuroendocrine cells found throughout the body. The exact cause is unclear, but there are several risk factors associated with these tumors.

Risk factors for neuroendocrine carcinoid tumors include:

  • Age: Neuroendocrine carcinoid tumors are more likely to be found in older adults. They are rare in children.
  • Sex assigned at birth: Females are more likely to develop neuroendocrine carcinoid tumors than males.
  • Family history of multiple endocrine neoplasia 1, neurofibromatosis type 1, or von Hippel-Lindau syndrome
  • Race: People who are Black are mor likely to develop GI carcinoid tumors than people who are white. People who are White are more likely to develop lung carcinoid tumors than other races.
  • History of conditions that affect the digestive fluids of the stomach, such as atrophic gastritis, pernicious anemia, or Zollinger-Ellison syndrome

It's important to note that these risk factors do not guarantee the development of neuroendocrine carcinoid tumors. If you have concerns about your risk or symptoms, it's best to consult with a healthcare professional for a thorough evaluation and personalized advice.

Symptoms

Symptoms of neuroendocrine carcinoid tumors may depend on the location of the tumor. The following are symptoms associated to different types of neuroendocrine carcinoid tumors:

  • Gastrointestinal tract neuroendocrine carcincoid tumors:
    • Stomach or abdominal pain or discomfort
    • Diarrhea
    • Nausea and vomiting
    • Inability to pass stool (intestinal blockage)
    • Blood in stool or change in bowel habits
    • Rectal bleeding or pain
    • Weight loss
    • Weakness
  • Lung neuroendocrine tumors:
    • Wheezing
    • Chest pain
    • Coughing with blood
    • Shortness of breath
  • Hormone producing tumors:
    • Facial and other skin flushing
    • Skin rash
    • Diarrhea
    • Shakiness
    • Increased thirst
    • Frequent urination
    • Sweating
    • High blood pressure
    • Rapid heart rate

As the tumors progress and spread to other parts of the body, additional symptoms may occur, such as:

  • Abdominal pain and bloating
  • Jaundice (yellowing of the skin or eyes)
  • Loss of appetite
  • Heart murmur

It's important to note that not all individuals will experience the same symptoms, and the severity and progression of symptoms can vary. If you have concerns about neuroendocrine carcinoid tumors or are experiencing any of these symptoms, it's best to consult with your healthcare professional for an accurate diagnosis and appropriate management.

Diagnosis

To diagnose neuroendocrine carcinoid tumors, doctors may perform the following examinations, tests, and procedures:

  • Medical history: Gathering information about your past and current health conditions, including any previous diagnoses.
  • Physical exam: A thorough examination of the body to check for any signs of disease or abnormalities, such as swollen lymph nodes.
  • Blood tests: Checking the levels of certain substances in the blood, such as hormones, that may indicate the presence of a tumor.
  • Urine tests: Measuring the amounts of specific substances in the urine, such as 5-HIAA or serotonin, which can be elevated in neuroendocrine tumors.
  • Tumor marker test: Checking for specific substances made by organs, tissues, or tumor cells that can indicate the presence or progression of neuroendocrine tumors.
  • Imaging tests: Using various imaging techniques like ultrasound, CT scan, MRI, PET scan, or octreotide scan to visualize any tumors or abnormal growths in the body.
  • Biopsy: Taking a small tissue sample from a suspected tumor to examine it under a microscope for cancer cells. This may be done using scopes, or thin tubes with a light and camera. The tube may be inserted into the lungs (bronchoscopy), esophagus (endoscopy), or rectum (colonoscopy).

It's important to consult with your doctor about which specific examinations, tests, and procedures are appropriate for your individual situation.

Treatment Options

The treatment goals are to remove or shrink the tumor or slow down tumor growth, control symptoms, and prevent complications.

Treatment options for neuroendocrine carcinoid tumors include:

  • Surgery: Surgery is the primary treatment option for localized tumors. It involves removing the tumor and surrounding tissue to prevent further spread. In some cases, endoscopic surgery may be suitable depending on the tumor's location, size, and malignancy degree.
  • Chemotherapy: Chemotherapy uses drugs to kill tumor cells. It may be used for advanced cases where the tumor cannot be removed by surgery.
  • Targeted therapy: Peptide receptor radionuclide therapy (PRRT) is a targeted therapy that can be used for neuroendocrine tumors. It specifically targets neuroendocrine tumor cells by attaching radioactive particles to them, which can kill the cancer cells.
  • Radiation therapy: This therapy uses energy beams, such as X-ray or proton beams, to kill tumor cells. Radiation therapy may be used if surgery is not an option.
  • Somatostatin analogs: Medications such as octreotide or lanreotide, are commonly used. They help reduce hormone production and alleviate symptoms associated with carcinoid syndrome, improving quality of life. These medications are typically administered as injections every 28 days.

Other treatments may be recommended if neuroendocrine carcinoid tumor has spread and is affecting other organs. It's important to note that each person's treatment plan will be unique and should be determined by an expert in neuroendocrine tumor treatment based on individual factors. Always consult with a healthcare professional for personalized advice.