What is Neuroendocrine Tumor & How is it Treated?|Causes, Symptoms, Prognosis of Neuroendocrine Tumor

To understand a Neuroendocrine Tumor, it is important to understand neuroendocrine system. The Neuroendocrine System consists of nerves and cells of glands which produce and release hormones in the bloodstream required by the body for normal functioning. Normally the cells of the gland in a Neuroendocrine Tumor belong to the endocrine system, although the neuroendocrine cells are spread throughout the body to include the lungs, pancreas, bowels, and stomach.

Any tumor resulting from uncontrollable cell growth of the neuroendocrine system is termed as a Neuroendocrine Tumor. These tumors can be both benign as well as malignant. The characteristics of Neuroendocrine Tumors are that they tend to grow very slowly and it may take several years for the first symptoms of a Neuroendocrine Tumor to develop. This is probably the reason behind a delayed diagnosis of a Neuroendocrine Tumor.

Neuroendocrine tumors are classified based on the location of the tumor and include:

Carcinoid Tumors: These are the type of Neuroendocrine Tumor that develops in the lungs.

Pancreatic Neuroendocrine Tumors: These tumors develop; in the pancreas.

Small/Large Bowel Neuroendocrine Tumors: These types of neuroendocrine tumors develop in the bowels whether it be the small or the large bowel.

Gastric Neuroendocrine Tumors: These tumors develop in the stomach.

Once an individual is diagnosed with a Neuroendocrine Tumor, then it becomes vital for the individual to consult with a physician to identify the location and type of the tumor and find out whether it is a benign or a malignant tumor so that aggressive treatment can be started at the earliest possible time for treating Neuroendocrine Tumor.

The root cause of a Neuroendocrine Tumor has not been identified as of yet but researchers have come up with certain risk factors which increase the chances of an individual developing a Neuroendocrine Tumor. These risk factors include:

Genetic Makeup & Family: The genetic makeup of an individual plays an important role in determining whether that individual is at risk for developing a Neuroendocrine Tumor.

Individuals with a family history of rare conditions like Multiple Endocrine Neoplasia Type 1 have a high chance of developing a Neuroendocrine Tumor. Similarly individuals with a family history of Von Hippel-Lindau syndrome also are at increased risk for developing a Neuroendocrine Tumor.

In some cases, if an individual has a family history of this condition then the chances of that individual is quite high of developing a Neuroendocrine Tumor.

Tobacco Abuse: Chronic smokers are also at an increased risk for developing a Neuroendocrine Tumor.

The symptoms of a Neuroendocrine Tumor depend on the organ that is involved. Some types of Neuroendocrine Tumor are completely asymptomatic while other forms of this condition produce a variety of symptoms. Some of the symptoms that an individual with Neuroendocrine Tumor will exhibit are:

• Fluctuations in blood sugars where sometimes they may be very high while at other times they may be too low.
• Persistent diarrhea with pain at the location of the tumor
• Unintentional weight loss
• Poor appetite
• Persistent cough with hoarseness of voice
• The presence of a lump in any part of the body
• Alteration in bowel and bladder pattern
• Jaundice in some cases
• Fever with night sweats
• Anxiety
• Frequent bouts of intense headaches

Chromogranin A Levels: Chromogranin A is a protein that is found in the neuroendocrine cells. To diagnose Neuroendocrine Tumors, a blood test to check the level of Chromogranin A is done. The higher the level of this protein when compared to other hormones like gastrin and insulin will clearly point towards a diagnosis of a Neuroendocrine Tumor.

Somatostatin Receptor Scintigraphy: This is a scan that is done to identify small pancreatic tumors that tend to arise as a result of Neuroendocrine Tumor.

Imaging: Advanced imaging studies in the form of a CT or an MRI scan can also be done to identify the location and the extent of a Neuroendocrine Tumor.

Biopsy: A tissue biopsy of the affected lump or area is taken which will clearly show cancer cells confirming the diagnosis of a Neuroendocrine Tumor.

The treatment options for a Neuroendocrine Tumor depend on various factors to include the type and location of the tumor and whether the tumor is benign or malignant. The age and overall health of the patient also plays a key role in formulating a treatment plan for Neuroendocrine Tumor.

For cases of benign tumors, observation is the first step where the tumor will be monitored with serial imaging to observe any change in the characteristics of the tumor. A surgery may be recommended if the size of the tumor may be large enough to cause symptoms and is a source of discomfort for the patient. Once the benign tumor has been removed then the patient may be called for followups to see for any recurrence of the tumor.

In cases of malignant Neuroendocrine Tumors, again surgery is the preferred route to go depending on the health status of the patient and the location of the tumor. The surgeon will remove the tumor along with some healthy tissues known as margins. The risks and side effects of the surgery involved will be discussed in detail with the patient before a consent form is signed.

In case if the location of the tumor is such that it cannot be removed, then alternate means of treatment are used which are basically radiation followed by chemotherapy.

In some cases, radiation and chemotherapy is followed after a surgery to completely eliminate all cancer cells from the affected area. This is normally done for Merkel cell cancer which is a form of a Neuroendocrine Tumor.

The prognosis of a Neuroendocrine Tumor depends on the following factors:

The stage of the tumor and whether the tumor is benign or malignant determined whether the prognosis of an individual with Neuroendocrine Tumor is good or not. If the cancer has spread to other parts of the body then the prognosis of the individual may be guarded to poor. However, if the tumor is identified early then the prognosis for Neuroendocrine Tumor is quite good for a successful outcome.

Another factor which plays a key role in determining the prognosis of an individual with Neuroendocrine Tumor is the level of differentiation. The level of differentiation in termed as the difference observed between the cancer cells and the normal cells of the body. If there is marked difference between these two cells then the prognosis is quite good. However if the differentiation is poor then the prognosis for neuroendocrine tumor becomes guarded to poor.

The grade of a Neuroendocrine Tumor also plays a factor in the prognosis of a Neuroendocrine Tumor. If the cancer cells are growing aggressively then the prognosis in such cases is poor when compared to cancer cells which are growing slowly.

The levels of Chromogranin A hormones play a vital role in deciding the prognosis of an individual with Neuroendocrine Tumor. The higher the level of the hormone the poorer will be the prognosis of an individual with Neuroendocrine Tumor.

• Steve Jobs, Founder of Apple
• Willko Johnson, A Well Known R&B Musician
• Nick Robinson BBC political reporter
• Dave Thomas the founder of Wendy’s Hamburger Chain had a Neuroendocrine Tumour but many newspaper reports said he died of liver cancer
• Siri Co-Founder Dag Kittlaus
• Recently, famous Indian actor Irfan Khan has been diagnosed as having Neuroendocrine Tumor.