1. Somatostatinomas Cause Many Symptoms
A somatostatinoma is a rare endocrine tumor usually found on the pancreas or small intestine. When the tumor is on the duodenum, the first part of the small intestine, it usually means that the person has von Recklinghausen's disease, also known as neurofibromatosis, a genetic disorder that affects the nervous system. A somatostatinoma secretes excessive amounts of a hormone known as somatostatin. Somatostatin is a chemical in the body that acts as a neurotransmitter. It main function is to inhibit certain functions in the body, including the release of certain hormones, acid secretion and intestinal absorption. Because this chemical acts on a number of systems in the body, there are many symptoms of somatostatinoma syndrome. These include weight loss, diabetes mellitus, gallbladder disease, low levels of hydrochloric acid in the stomach which cause poor digestion, diarrhea and fat in the feces and weight loss. These symptoms occur more frequently if the tumor is located on the pancreas than if it is located on the small intestine.
2. Uncommon Malady Found by Accident
Somatostatins are very uncommon; each year, about one in 40 million people are diagnosed with one. For this reason, most somatostatinomas are found by accident during a cholecystectomy--removal of the gallbladder--or during an endoscopy. When you go to your doctor, she will order a fasting blood test that measures your somatostatin level. Because this condition is very rare, it is only done at certain centers. A high level of somatostatin in the blood is necessary to confirm the diagnosis the presence of a somatostatinoma. Once it is determined that these levels are high, a number of other tests might be used to find the exact position of and stage one of these tumors, including spiral CT scanning, MRIs, and somatostatin receptor scintigraphy, and endoscopic ultrasound. These cancerous tumors are usually pretty large--about 1 1/2 to 2 inches in diameter--and they frequently metastasize to the liver. In fact, by the time the somatostatinoma is discovered it has usually spread to the liver. Treatment can include surgery, chemotherapy or radiation therapy.
3. A Middle-age Phenomenon
Most patients who are diagnosed with this disease are between 40 and 60 years of age--the average age is 51, and it is twice as common in women as in men. Like any cancers, somatostatinoma can have genetic or environmental causes.
