What and Why Neuroendocrine Tumors?
These are neoplasms. These arise from cells of endocrine system and nervous system. They may be of benign or malignant in nature. They are heterogeneous group of neoplasms. Majority of neuroendocrine tumors are sporadic but some may have genetic component. These are slow growing tumors. These tumors can secret hormones and peptides. They can occur in any part of the body but most commonly occur in intestine, pancreas, and respiratory system.
Classification of Neuroendocrine Tumors
World Health Organization (WHO) classified neuroendocrine tumors into three categories.
These categories tell us about the grade of tumor.
• Well-differentiated (low grade) neuroendocrine tumors, cells of tumor are slow growing
• Well-differentiated (intermediate grade) neuroendocrine tumors, cells are growing and
dividing quickly than normal cells
• Poorly differentiated (high grade) neuroendocrine tumors, cells of tumor are fast
Anatomic distribution of Neuroendocrine
Neuroendocrine tumors have also been classified according to their anatomic site of origin.Most often these are located in the intestine, pancreas, or the respiratory system. Mostly these are distributed in the Kulchitsky cells or enterochromaffin-like cells.
Signs and symptoms of Neuroendocrine
Signs and symptoms depend on the size, type, and location of the tumor. Most common signs and symptoms are:
Flushing, diarrhea, asthma or wheezing, abdominal cramping, peripheral edema, heart
palpitations, bronchospasm, tachycardia, fluctuating blood pressure, pellagra, dermatitis,
dementia, acromegaly, and hemorrhage are the symptoms.
• Multiple endocrine neoplasia type 1 (MEN1)
• Multiple endocrine neoplasia type 2 (MEN2)
• Von Hippel-Lindau disease
• Neurofibromatosis type 1
• Tuberous sclerosis
• Carney complex
The Main types of neuroendocrine
Two main types of neuroendocrine tumors:
1. Carcinoid tumors – arise in the digestive tract, lungs, thymus, lymph nodes, brain, bone,
gonads, or skin.
2. Pancreatic neuroendocrine tumors – arise in the pancreas.
Cells that are responsible for neuroendocrine tumors can produce hormones so the tumor can also produce hormones. These tumors can be benign or malignant, non-cancerous or
cancerous. They grow slowly but some forms of neuroendocrine tumors are fast growing.
Survival rate of neuroendocrine tumors
The survival rate of five year is approximately 75% in patients with localized disease, 72% in patients with spread to regional lymph node metastases and 20-43% in patients with distant metastasis.
• Inherited syndromes. Multiple endocrine neoplasia type 1, Hippel-Lindau syndrome,
neurofibromatosis, multiple endocrine neoplasia type 2, tuberous sclerosis complex.
• Race/ethnicity. In general, NETs are common in white people. The location where NETs begins
• Gender. Neuroendocrine tumors are more common in women.
• Age. NETs occur in any age but in children it is rare.
• Other medical conditions. People with stomach diseases are at greater risk of developing
specific types of NETs.
• Environment and diet. There is connection between environment and diet with
Questions to ask
➢ Where is tumor located?
➢ What is the stage of the tumor?
➢ What is the grade of tumor?
➢ Do I have carcinoid syndrome?
➢ What are my treatment options?
➢ What are the side effects of the treatment?
How to Diagnosis Neuroendocrine Tumors
Most NETs are found unexpectedly when people have medical procedures done for some other
reasons. Following tests may be used to diagnose a NET.
• Biopsy. Removal of small amount of tissue for examination to make a diagnosis of NET.
Biopsy of tumors like pheochromocytoma is life threatening so such tumors should not
• Blood/urine tests. Take samples of blood and urine to check abnormal levels of
hormones. Urine tests check the level of 5-HIAA. Blood test check the level of a protein,
• Molecular testing of the tumor. To identify specific genes, proteins, and other factors
important for tumor.
• Endoscopy. It allows seeing inside the body. The person is sedated before this
procedure. If there is any abnormality biopsy will be performed.
• Ultrasound. It uses sound waves to create picture of internal organs. Tumors generate
echoes of sound waves. Different types of ultrasounds can be used.
• X-ray. It also create image of internal organs using less radiations. It is not always useful.
Barium x -ray are mostly used.
• Computed tomography scan. It takes pictures of internal organ using x-ray from
different angles. IT creates a 3-dimensional image. It can be used to measure tumor’s
size. For better details a contrast medium is used. Dye (contrast medium) is injected into
• Magnetic resonance imaging. IT uses magnetic fields to make images of the body. IT can
also be used to measure tumor’s size.
• Nuclear medicine imaging. A small amount of radioactive drug is injected to patient’s
vein. The body is then scanned to see the radioactivity.
• Octreoscan. It uses octreotide with indium-111. This is useful to see where the tumor
• Positron emission tomography. Traces used in PET scan include gallium-68 and
fluorodeoxyglucose. It is mostly used for grade 3 NETs.
Treatment Neuroendocrine Tumors Factores
Treatment depends on several factors such as:
▪ The type of NET
▪ Stage and grade
▪ Possible side effects
▪ The patient’s preferences and health
Types of treatment:
❖ Surgery. Removal of tumor and surrounding healthy tissue. Completely removing the
tumor is best treatment. Before surgery the anesthesiology team must be aware of the
❖ Somatostatin analogs. It is a hormone that control release of insulin and glucagon.
These are drugs used to control symptoms caused by NETs. Somatostatin analogs
include octreotide (Sandostatin) and lanreotide (Somatuline Depot).
❖ Chemotherapy. It destroys cancer cells. Chemotherapy uses 1 drug or combination of
drugs at same time. Side effects of chemotherapy include fatigue, infection, nausea,
vomiting, hair loss, loss of appetite, diarrhea.
❖ Immunotherapy. It boosts the body’s natural defenses to fight against tumor. Interferon
helps the body’s immune system to work better.
❖ Peptide receptor radionuclide therapy. It is a radioactive drug that binds to cell’s
Somatostatin receptor. It enters the cell, and damage the tumor.
❖ Radiation therapy. External-beam radiation is the most common type of treatment.
❖ Liver-directed treatment.
a) Radiofrequency ablation
b) Hepatic artery embolization
c) Radio embolization
o Watching for recurrence. Tumor recurs when some abnormal cells remain undetected.
o Managing long-term side effects. Talk to doctor about long term side effects.
o Keeping health records. Work to develop follow-Up
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