Posted On Dec 23, 2019

Department of

Manipal Hospitals

Neuroendocrine cancer is a rare form of cancer, but one which has gained a lot of coverage in the news in the recent times, with a prominent Bollywood actor reportedly being afflicted with this condition.

Endocrine cells are those that produce and release hormones. A neuroendocrine tumor originates in the body’s neuroendocrine system which consists of the hormone-producing endocrine cells and the nerve cells. This tumor can develop in different parts of the body such as the brain, lungs and the gastrointestinal tract including the stomach and the intestines. The tumor may be malignant or benign depending on whether or not the growth has affected other parts of the body.

Types of Neuroendocrine Tumor (NET)

Depending on the particular organ affected, there are different types of NET. They are:

  1. Pheochromocytoma: It is a rare tumor that begins in the cells of the adrenal gland. These cells release the adrenaline hormone during times of stress. A tumor here increases the production of the hormones adrenaline and noradrenaline, which increases the blood pressure and the heart rate. Usually, this kind of tumor is benign, but it is still dangerous as it can release large amounts of adrenaline into the bloodstream after an injury.
  2. Merkel Cell Cancer: It is a rare, aggressive skin cancer which grows rapidly. It starts in the cells just beneath the parts of the skin which are exposed to a lot of sunlight, such as the head, neck, arms and legs, and in the hair follicles.
  3. Neuroendocrine Carcinoma: This kind of NET develops in other areas of the body such as the gastrointestinal tracts, lungs and the brain. It occurs in about 60% of the cases.
  4. Paraganglioma: It originates in some nerve cells that are spread throughout the body. It usually affects people between the ages of 30 and 50. It is slow-growing and can be benign or cancerous. However, if left untreated, this can result in a severe damage which may not be rectified even through surgery.


The common signs and symptoms of NET depend on the location of the tumor.

  • If the tumor is located in the intestines, the symptoms are:
    • Chronic diarrhea resulting in weight loss
    • High blood pressure
    • Fatigue
    • Nausea and vomiting
    • Bleeding
    • Black stools
  • In case of tumors located in the lungs, the patient may experience the following symptoms:
    • Cough
    • Chest pain
    • Bleeding in the sputum
  • If the tumor originates in the skin (Merkel cell cancer):
    • Painless, hard and shiny lumps on the skin that can be of various colors
  • Symptoms of Pheochromocytoma:
    • Fever
    • Headaches
    • Nausea
    • Vomiting
    • Rapid pulse
    • High blood pressure
    • Sweating
    • Palpitations in the heart
  • Symptoms of neuroendocrine carcinoma:
    • High blood glucose levels (hyperglycemia) resulting in frequent urination and increased thirst and hunger.
    • Low blood glucose levels (hypoglycemia) causing fatigue, dizziness, sweating, seizures and fainting
    • Persistent pain in a specific part of the body
    • Loss of appetite
    • Gastric ulcer disease
    • Jaundice
    • Formation of lumps in any part of the body
    • Skin rashes


Diagnosis and Treatment:

While diagnosing NET, your doctor takes into account a few factors such as the type of tumor suspected, the symptoms observed, age and medical condition. All diagnostic tests may not necessarily be conducted on the patient. The common diagnostic techniques are:

  • Physical Examination: The doctor looks for visible signs of NET, such as lumps, skin coloration, clammy skin etc.
  • Blood and Urine Tests: The doctor may collect the blood and urine samples in order to test for abnormal levels of hormones and increased levels of adrenaline.
  • Magnetic Resonance Imaging (MRI): It is a scanning technique which doesn’t use radiation, but instead, uses magnetic fields to view a tumor and measure its size. A special dye called contrast medium is injected into the patient’s vein before the scan in order to get a clearer image.
  • Computed Tomography (CT) Scans: It uses X-rays to get more detailed images of sections of the body than the regular X-rays.
  • Biopsy: It involves removing a tiny portion of the tumor cells or tissues so it can be examined under a microscope.
  • Endoscopy: A procedure in which the doctor uses specialized equipment to view the tumor.
  • Positron Emission Tomography (PET-CT) Scan: A small amount of radioactive substance is injected into the patient’s body, which is then absorbed by the cancer cells. A scanner is used to detect the presence and concentration of this radioactive substance in order to produce images of the interior of the body.

NETs are especially difficult to diagnose due to the vague symptoms which present themselves in people affected with this disease. There is no cure for neuroendocrine cancer but it can be controlled if detected in the early stages. Some of the treatment measures available are:

  • Surgery: This is the first line of defence. The tumor along with a portion of the surrounding healthy cells, called a margin, is removed surgically. Through laparoscopy, which is a modern surgical technique involving making small incisions instead of wide cuts in the body, the chances as well as period of recovery is significantly improved.
  • Medications: These include medicine needed to kill the cancer cells which still remain in the body after the surgery, and targeted treatments which attack the precise genetic material found in the tumor. Biologic drugs boost the immune system and this technique is called immunotherapy.
  • Radiofrequency Ablation: Radiofrequency waves are used to increase the temperature within the tumor tissue and break it down.
  • Radiation Therapy: It is generally resorted to when the NET is in a location that makes the surgery very difficult. High-energy X-rays are used to destroy the cancer cells. A radiation therapy regimen consists of a specific number of treatments given over a fixed period of time.
  • Chemotherapy: Drugs which make the tumor cells unable to grow and divide are administered by a medical oncologist. Usually the drugs are administered through an intravenous tube inserted into a vein or in the form of a capsule or a pill.
  • Nutritional Treatment: In some cases, changing the diet can help ease or prevent the symptoms.
  • Palliative Therapy: This is form of treatment that focuses on alleviating patient suffering.
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