VIPoma

The Verner - Morrison syndrome or Water Diarrhea Hypokalemia achlorhydria ( WDHA ) (According to the vasoactive intestinal peptide also VIPoma ) is an adenoma or (more often ) adenocarcinoma, which starts from the D1 - cells of the pancreas and is one of the neuroendocrine tumors. Approximately one in ten tumor, however, is not primarily in the pancreas. The first description was by Verner and Morrison in 1958.

Epidemiology

The VIPoma is a very rare disease. Occurs every year about a case on 10 million people. Cases have been described in adults and in children. The peak age is around 50 years of age.

Symptoms

The adenoma secretes the vasointestinale peptide that stimulates mainly the secretion of the intestinal mucosa. Leading symptom is therefore a massive diarrhea with watery stools and a chair volume over three liters per day ( according to some reports, up to 20 liters). This meant that is descriptive also spoken of " pancreatic cholera ". Hypokalemia ( by the enteral potassium loss ) and achlorhydria part of the symptoms. Since the VIP probably also has a glucagon -like effect, it often leads to a slightly diabetic metabolism. Hypercalcemia and hypophosphatemia may be added. As with a carcinoid flush ( attacks of redness of the face and neck) can occur. Because of the symptoms is commonly known as ' WDHH syndrome ' ( Watery diarrhea, hypokalemia, Hypo-/Achlorhydrie )

The diagnosis is based on the clinical picture and the high VIP level in the serum.

Treatment

As pharmacological treatment option is octreotide, an analogue of somatostatin, are available. It inhibits the release of VIP, thus limiting the symptoms. Non- metastatic tumors can be surgically removed. In the presence of metastases (usually in the liver ) is a surgical procedure is usually no longer make sense. Then, however, a partial remission can often be achieved with systemic chemotherapy.

Forecast

Due to the massive dehydration, the disease can run quite deadly. More than half of the patients have no distant metastases and can be definitely cured by surgical intervention at the time of diagnosis.