Verner-Morrison syndrome is characterized by an extremely rare malignant tumor of the pancreas. The tumor produces an increased amount of a hormone also known as a vasoactive intestinal peptide. The main symptom of the disease is severe, stubborn diarrhea, which can also have fatal consequences due to the extreme loss of water.
What is Verner-Morrison Syndrome?
Verner-Morrison syndrome is a very rare neuroendocrine tumor of the pancreas. It is a tumor of the neuroendocrine system, which includes the nervous and endocrine systems. This tumor is also known as a VIPoma because it produces an increased amount of the hormone VIP. For all you need to know about methanol poisoning, please visit phonecations.com.
VIP is the abbreviation for Vasoactive Intestinal Peptide. This hormone consists of 28 amino acids and serves as a neurotransmitter in the central nervous system. It relaxes the smooth muscles of several organs. These include the bronchi, trachea, stomach or intestines. At the same time, it is also an important vasodilator, which means that it is also responsible for dilating blood vessels.
When the muscles in the stomach and intestines relax, water is released in the small intestine. In addition, the production of hydrochloric acid in the stomach is restricted. This causes massive diarrhea that is similar to cholera. The symptom of lack of stomach acid is called achlorhydria. Overall, Verner-Morrison syndrome affects only one in ten million people.
The tumor affects 90 percent of the pancreas. In the remaining ten percent of those affected, it is located in the sympathetic trunk of the spinal cord, in the adrenal glands or in the bronchi. The prognosis of the disease depends on whether metastases have already formed.
Causes
Little is known about the causes of Verner-Morrison syndrome. It is the malignant degeneration of D1 cells in the pancreas. The D1 cells are responsible for producing the VIP hormone. Under physiological conditions, VIP synthesis is subject to hormonal regulation mechanisms. Among other things, the growth hormone has an inhibiting effect on the formation of VIP.
However, the tumor produces the hormone largely independently and autonomously of these regulatory mechanisms. The VIP hormone is a neurotransmitter that is responsible for signal transmission in the central nervous system. As part of this function, it inhibits the production of gastric acid and increases the formation of the basic HCO3 anion. The result is an increased secretion of water from the small intestine.
If this hormone is produced in large quantities, the water excretion in the intestine is so high that those affected lose an average of three liters of water a day via the intestine. In extreme exceptional cases, up to 20 liters of water can also be excreted. As a result, there is a risk of dehydration of the body and a severe loss of potassium.
Furthermore, the hormone VIP has an inhibitory effect on gastric acid production. It also supports insulin’s hormonal opponent, glucagon, in raising blood sugar levels. In the long term, diabetes mellitus can also occur.
Symptoms, Ailments & Signs
The characteristic symptom of Verner-Morrison syndrome is massive and persistent diarrhea that is similar to cholera. The large loss of fluids from the body and the hypokalemia cause abdominal cramps and states of confusion in addition to diarrhea.
Other symptoms include muscle cramps, severe tiredness, vomiting and cardiac arrhythmias. The disease can be fatal. Here, the loss of fluid is the greatest risk factor. In some cases, metastases form in the liver. When metastases have started to form, the prognosis of the disease becomes less favorable.
Diagnosis & course of disease
Diagnosing Verner-Morrison syndrome is difficult because the condition is so rare. The suspicion of VIPoma is often not mentioned at all. However, if there is persistent and massive diarrhea that does not go away, Verner-Morrison syndrome should also be considered. The determination of chromogranin A also provides valuable information.
Because this substance is produced by all neuroendocrine tumors. If the suspected diagnosis is now available, blood tests for the hormone VIP are carried out. If the VIP level is very high, this gives a strong indication of a VIPoma. The determination of chromogranin A also provides valuable information. Because this substance is produced by all neuroendocrine tumors. The tumor can then be easily detected by imaging methods such as CT and MRI.
Complications
In Verner-Morrison syndrome, those affected primarily suffer from very severe diarrhea. This has a very negative effect on the patient’s quality of life and makes everyday life considerably more difficult. As a rule, those affected lose a lot of fluids as a result, which in turn can promote severe dehydration and deficiency symptoms if the nutrients are not substituted elsewhere.
There is also cramping in the muscles and a sedation. In general, those affected suffer from impaired consciousness and can also lose consciousness completely. As it progresses, Verner-Morrison syndrome leads to vomiting and heart problems. If left untreated, the disease can also lead to the death of the patient. Metastases form in the liver, which can eventually lead to cancer in other regions.
There are no other complications in the treatment of Verner-Morrison syndrome. Depending on the patient’s condition, the tumor may be removed. However, complete healing does not occur, so that the life expectancy of the patient is significantly reduced due to Verner-Morrison syndrome. There are usually severe side effects, especially when chemotherapy is used.
When should you go to the doctor?
Since Verner-Morrison syndrome cannot heal on its own, those affected should definitely consult a doctor. The sooner treatment is given, the better the further course of the disease. In the worst case, the person concerned dies as a result of Verner-Morrison syndrome if this is not treated. A doctor should be contacted for this disease if the patient suffers from persistent diarrhea. This diarrhea cannot be stopped even with ordinary medication and occurs without any particular reason.
Furthermore, there is also permanent confusion or severe cramps in the muscles, which also indicate the disease. In some cases, those affected also suffer from heart problems or pain in the heart. If the symptoms of Verner-Morrison syndrome occur, the person concerned should contact an internist or a general practitioner. Further treatment is usually carried out in a hospital by a specialist. The disease may lead to a significantly reduced life expectancy, so that psychological treatment would also make sense.
Treatment & Therapy
Therapy for Verner-Morrison syndrome depends on the condition of the tumor. If the tumor is detected early, there is a good chance that metastases have not yet formed. In this case, a complete cure can be achieved by surgically removing the tumor. If metastases are already present, an operation no longer makes sense.
Then there is the option of treating the tumor with chemotherapy. However, the success of chemotherapy is extremely questionable. However, the growth of the cancer can be stopped for a while. The two drugs ocreotide and streptozotocin, among others, are available for drug treatment of a VIPoma. Ocreotide works in a similar way to the growth hormone somastatin.
It inhibits the production of the book salivary hormones. Streptozotocin again shows a different mode of action. This substance inhibits the cells of the pancreas. Only less VIP will be produced. While these drugs cannot cure the disease, they are a good way to slow the growth of the tumor. Chemotherapy is carried out with cytostatics such as 5-fluorouracil, which disrupts the build-up of nucleic acids.
Prevention
There is no recommendation for prevention of Verner-Morrison syndrome. The causes of the degeneration of the D1 cells are not yet known.
Aftercare
Follow-up care for Verner-Morrison syndrome depends on the therapy. Rest and relaxation are important after an operation. A further physical examination takes place during the follow-up visit. Imaging procedures are used to determine whether further metastases are present. If the tumors do not metastasize and do not recur, no further follow-up is necessary.
The patient only has to make use of the check-up appointments that are typical for cancer diseases. These usually take place every three months at first, then every six months and later every two years. Further follow-up measures depend on the individual course of the disease. As part of the aftercare, any prescribed painkillers and other preparations must be discontinued or carefully tapered off.
This depends on the patient’s state of health and the course of the disease. Several follow-up appointments are usually necessary before the medication can be completely discontinued and the patient can be discharged. Comprehensive follow-up care is required after chemotherapy.
The patient sometimes needs therapeutic support. Again, bed rest and rest are important. In addition, any consequences of the chemotherapy are clarified. Depending on the results of the examinations, further treatment measures may then be necessary. Aftercare for Verner-Morrison syndrome is carried out by the responsible specialist. After an operation, the surgeon or chief physician is responsible.
You can do that yourself
Verner-Morrison syndrome requires medical treatment in the first place. The treatment of the malignant tumor can be supported by a number of self-help measures.
First of all it is important to avoid stress. Patients should rest extensively and otherwise comply with the doctor’s instructions. These usually also affect the intake of sufficient liquid, since the tumor leads to severe dehydration. Diet should also be changed. A bland diet is recommended, which may need to be supplemented by dietary supplements. If pain or other discomfort occurs, the doctor must be informed. Sporting activity should be avoided during the acute phase of the disease. After surgery, moderate exercise can aid in healing.
However, due to its location in the stomach, Verner-Morrison syndrome has a negative impact on gastric acid production. This can lead to persistent gastrointestinal problems. The diet should therefore be maintained beyond the treatment. The doctor in charge can consult a nutritionist, who can provide further tips and measures. With early treatment, supported by the self-help measures mentioned, the tumor disease subsides completely in many cases.
