Verner-Morrison Syndrome
| Classification according to ICD-10 | |
|---|---|
| C25.4 | Malignant neoplasm of the pancreas (endocrine part) |
| E16.8 | Increased secretion of VIP |
| ICD-10 online (WHO version 2019) | |
The Verner-Morrison syndrome (English water diarrhea hypokalemia achlorhydria , WDHA ), based on the vasoactive intestinal peptide also VIPoma , is an adenoma or (more often) adenocarcinoma that originates from the D 1 cells of the pancreas and to the neuroendocrine tumors heard. However, about every tenth tumor is not primarily in the pancreas. It was first described by Verner and Morrison in 1958.
Epidemiology
VIPoma is a very rare disease. There is about one case in 10 million people each year. Cases have been reported in adults and children. The maximum age is around the age of 50.
Symptoms
The adenoma secretes the vasointestinal peptide (VIP), which mainly stimulates the secretion of the intestinal mucosa. The leading symptom is therefore massive diarrhea with watery stools and a stool volume of over three liters per day (according to some reports up to 20 liters). This led to the fact that “pancreatic cholera ” is also used descriptively . Hypokalemia (due to the enteral loss of potassium ) and achlorhydria are part of the clinical picture. Since the VIP probably also has a glucagon- like effect, it often leads to a slightly diabetic metabolic situation. Hypercalcaemia and hypophosphataemia can also occur. As with the carcinoid , a flush (spasmodic reddening of the face and neck) may occur. Because of the symptoms is also called from, WDHH syndrome '( W ässrige D urchfälle, H ypokaliämie, H ypo- / achlorhydria)
The diagnosis is based on the clinical picture and the high VIP levels in the serum.
treatment
Octreotide , an analogue of somatostatin , is available as a pharmacological therapy option . It inhibits the distribution of VIP and thus limits the symptoms. Non-metastatic tumors can be surgically removed. If there are metastases (mostly in the liver ), an operative procedure is usually no longer useful. Then, however, partial remission can often be achieved with systemic chemotherapy .
forecast
Due to the massive dehydration , the disease can be fatal. More than half of the patients have no distant metastases at the time of diagnosis and can definitely be cured by surgery.
literature
- LJ DeGroot, JL Jameson: Endocrinology . 2006, ISBN 0-7216-0376-9 .
Individual evidence
- ^ JV Verner, AB Morrison: Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia. In: Am J Med. 1958; 25 (3), p. 374.
