Verner Morisson syndrome (VIPom), a tumor secreting a vasoactive intestinal peptide, is a very rare neuroendocrine neoplasm. VIPoma is most commonly located in the tail of the pancreas, but is also found in the adrenal glands, retroperitoneal space, mediastinum, lungs, and small intestine. The most characteristic symptom is profuse diarrhea. The prognosis depends on the stage of the diagnosis. How is Verner Morisson Syndrome treated?
Contents:
- Verner Morisson (VIPom) syndrome - symptoms
- Verner Morisson syndrome (VIPom) - diagnosis
- Verner Morisson syndrome (VIPom) - treatment
- Verner Morisson (VIPom) syndrome - prognosis
VIPomais a neoplasm characterized by the independent secretion of intestinal vasoactive peptide, which in the physiological state is secreted by the intestines and pancreas. The task of the vasoactive intestinal peptide is to stimulate the secretion of intestinal juice, dilate the blood vessels of the gastrointestinal tract and inhibit gastric motility and the secretion of gastric juice.
Under physiological conditions, vasoactive intestinal peptide is secreted by the passage of gastric acid into the duodenal lumen. In the case of a tumor secreting vasoactive intestinal peptide, this substance is secreted, regardless of the food consumed, in an uncontrolled manner, causing undesirable symptoms of the gastrointestinal tract.
VIPoma belongs to the group of malignant neoplasms that tend to form distant metastases.
VIPoma is an extremely rare cancer, affecting 1 / 10,000,000 people a year.
This tumor may be a component of the MEN1 syndrome, i.e. multiple endocrine neoplasia, then, apart from the VIPoma, there is primary hyperparathyroidism and a tumor of the anterior pituitary gland.
Verner Morisson (VIPom) syndrome - symptoms
The most characteristic symptom is very profuse watery diarrhea with a volume of about 20 liters per day. These diarrhea are persistent and persistent by starvation. As a result of profuse diarrhea, there is significant dehydration and related electrolyte disturbances. As a result, significant potassium loss occursmuscle weakness, abdominal muscle cramps, up to heart rhythm disturbances.
Nutritional deficiencies are also common, especially iron and vitamin B12 deficiency. Less typical symptoms include weakness, nausea, weight loss, gas, indigestion, rash, and reddening of the facial skin caused by vasoactive intestinal peptide expansion.
VIPoma causes clinical symptoms after reaching a certain size, therefore in 60% of cases at diagnosis distant metastases occur, most often in the liver, although they can also occur in the lungs and lymph nodes.
Sometimes symptoms may be due to the presence of metastases in distant organs. These include jaundice, shortness of breath, hemoptysis, and an enlarged liver.
Verner Morisson syndrome (VIPom) - diagnosis
Diagnosis is based on a medical examination, laboratory tests and imaging. Profuse diarrhea not related to the consumption of meals is the basis for laboratory tests.
There are markers specific for the tumor secreting vasoactive intestinal peptide, including blood VIP concentration. The result>200pg / ml is the basis for in-depth diagnostic imaging.
A marker not specific for VIPoma is the concentration of chromogranin A. Based on the result, it is possible to assess the stage of the tumor and whether it has developed distant metastases
Additionally, blood tests are performed to assess the level of electrolytes (electrolyte ionogram), which typically shows a reduced concentration of potassium and chlorine, and an increased concentration of calcium.
Increased blood glucose is also a common symptom. In order to assess the primary tumor and the stage of the disease, computed tomography and receptor scintigraphy are performed, in which metabolically active foci, most often located in the tail of the pancreas, are visible.
It is also important to perform a histopathological examination, thanks to which the type and stage of the neoplasm can be assessed.
Verner Morisson syndrome (VIPom) - treatment
Initially, the aim is to compensate for electrolyte disturbances caused by profuse diarrhea. Intravenous fluid therapy is often required.
The basic method of treatment is surgery to excise the tumor with a he althy tissue margin. Most often, diazoxide is used before surgery, which inhibits the occurrence of diarrhea, thanks to which it is possible to avoid electrolyte disturbances in the perioperative period.
In some cases it is also possibleremoval of individual liver metastases, if any.
In the case of inoperable tumors, local embolization of the tumor surface is used, thanks to which the blood vessels supplying blood to the tumor are closed and the tumor undergoes partial necrosis. Chemotherapy is not effective in treating VIPoma.
Verner Morisson (VIPom) syndrome - prognosis
The prognosis depends on the stage at which the tumor was diagnosed and whether the tumor is operable. It is assumed that if the VIPoma is removed successfully, the 5-year survival rate is 95%.
Read also: Neuroendocrine tumors (NETs): types. What are the symptoms of endocrine tumors?
About the authorBow. Agnieszka MichalakA graduate of the First Faculty of Medicine at the Medical University of Lublin. Currently a doctor during postgraduate internship. In the future, she plans to start a specialization in pediatric hematooncology. She is particularly interested in paediatrics, hematology and oncology.Read more articles by this author