Zollinger Ellison syndrome is a disorder that causes tumors in the pancreas or small intestine. It is a rare disorder that develops in people of age group 25-50 years. ZE syndrome causes tumors, also called as gastrinomas in the small intestine which in turn triggers the stomach to secrete excess of acid. Mild to moderate kind of ZE syndrome can be managed with medications that help in reducing stomach acid. Only in severe cases chemotherapy/radiation or surgery is required.
Process Of Digestion :
Anything that you put inside your mouth travels through the esophagus along with saliva and enters the stomach. The process of digestion begins right from your mouth wherein the food gets broken into small pieces and gets mixed with saliva to form a pulp. Several enzymes and digestive juices are secreted in the stomach where the food gets broken down further. After few minutes of action in the stomach, partially digested food particles would enter the duodenum (small tube found in small intestine).
The digestion process continues further in the duodenum where the food pulp gets mixed with insulin hormone produced by the pancreas. This hormone helps in maintaining blood sugar in the body by absorption of sugar molecules in the food and the liver helps in digesting fat molecules. Complete absorption of food takes place in the small intestine and leftover residue is pushed into the large intestine and gets expelled through the rectum.
Some of the major symptoms of ZE syndrome include diarrhea, stomach pain, nausea and vomiting and sudden burning pain in the upper abdomen. In due course, the affected person may feel fullness of stomach and bloated feeling. It can cause internal bleeding in the small intestine. For some people due to formation of tumor, weight loss would be there.
Causes Of Zollinger Ellison Syndrome :
ZE syndrome develops when an individual has tumor/tumors in his pancreas or small intestine. But what exactly triggers formation of tumor is still not clear. The location point of tumor is very crucial in interfering with the process of digestion. Small intestine is the place where the actual digestion and absorption takes place and it is the place where the digestive enzymes secreted by the liver, the pancreas and gallbladder mixes.
The digestion would be at its peak in the first part of small intestine and any tumor occurring in the intestine or pancreas can seriously interfere with the digestion. Further there is full scope for the tumor to metastasize to other parts of the intestine easily. The tumor largely affects the lymph nodes of the liver nearby.
Piling of dead cells with the healthy cells leads to tumor or gastrinomas which in turn triggers the stomach to produce more acid. This condition can lead to acidity and chest burn and sometimes peptic ulcer. Ulcer is formed when the stomach is continuously under the pressure of excess of acid.
Hereditary Factor :
A peculiar disorder known by name Multiple Endocrine Neoplasia shortly called as MEN 1 can be inherited from parents. Individuals with this disorder are likely to have tumors in thyroid glands or pituitary glands. Also they are likely to develop tumors in pancreas and intestine. Hence children whose parents are of MEN type 1 disorder are at increased risk of ZE syndrome.
ZE syndrome is a very rare phenomenon targeting 1 out of 1 million people.
After completing the physical examination, your doctor would listen to your symptoms and general health condition. He may order for tests like blood culture to check the gastrin levels and further order for secretin stimulation test. Blood test will be repeated again after taking a shot of secretin injection. If the results show high levels of gastrin it can be due to tumor.
Endoscopy may be done to get accurate picture of the duodenum and pancreas. A thin tube fitted with tiny camera and light would be inserted into your throat and sent into the stomach. Through this tubing, your doctor would gently remove tissue sample from the intestine to get it tested in the lab. Tumors in any part of the digestive tract can be detected with endoscopy. If required, imaging tests like CT or MRI scan would be done.
Treatment for Zollinger Ellison syndrome depends on the size of tumor, its intensity and growth and its location and grading. Before starting with treatment for tumor, symptoms of acidity should be controlled. The stomach would produce too much of acid owing to gastrinomas leading to peptic ulcer.
Medications are prescribed for managing acidity. Proton pump inhibitors like lansoprazole, omeprazole and pantoprazole with names like Prilosec or Protonix are prescribed in suitable strength. These drugs can control the secretion of acid by blocking the pumps that secrete acid and hence the name “Proton Pump Inhibitors”.
You should not take these drugs without consulting with your health care provider. Elderly people are likely to develop mild fractures on hip bone and spine if they take this medication for long.
- Surgery : Surgery is considered as last option for ZE syndrome if the patient fails to respond to other treatments. Small tumors are difficult to be identified and removed. An expert surgeon with advance tools can locate tiny tumors from the intestine. Removing part of pancreas due to tumor can inhibit insulin secretion and if part of liver is removed various complications can occur. Hence your doctor would outweigh the pros and cons before operating you surgically.
- Chemotherapy : Medications to slow down the growth of tumor are given either as oral pills or as injection. This can produce adverse effect and your doctor would explain about its outcome. Some of the common medications like doxorubicin or streptozotocin (Zanasar) are prescribed.
- Radiation : Here the patient will be asked to lie down on a table and high powered beam of light are passed directly into the regions of tumor for destroying them. In this process some of the healthy cells and tissues may also die.
- Outcome : Stage of cancer determines the outcome of result in any kind of cancer. For mild to moderate type of tumor medications can help in controlling the symptoms. Prognosis and rate of survival depends on the age of the patient, his/her health condition and intensity of tumor.