Overview of somatostatinoma
Somatostatinoma is a rare type of neuroendocrine tumour that grows in the pancreas and sometimes in the small intestine. A neuroendocrine tumour is made up of hormone-producing cells. The cells that produce this hormone are called islet cells.
A somatostatinoma develops exclusively in the delta eyelet cell, which is responsible for producing a hormone called somatostatin. Tumour cells make more of this hormone.
When your body produces excess somatostatin hormones, it stops the production of other pancreatic hormones. When those other hormones become deficient, they can eventually lead to symptoms.
Symptoms of a somatostatinoma
Somatostatinoma symptoms usually start mild and gradually increase in severity. These symptoms are similar to those caused by other medical conditions. For this reason, you must make an appointment with your doctor to get a proper diagnosis. This should ensure proper treatment for any medical conditions underlying your symptoms.
Symptoms may include the following:
- Abdominal pain (very common symptom)
- Unexplained weight loss
- Steatorrhea or fatty stools
- Intestinal obstruction
- Jaundice or yellow skin (more common when there is somatostatinoma in the small intestine).
Medical conditions other than somatostatinoma can cause many of these symptoms. This happens very often because these are very rare. However, only your doctor can diagnose the exact condition behind your specific symptoms.
Causes and risk factors of somatostatinomas
The cause is currently unknown. However, some risk factors can lead to a somatostatinoma.
This condition, which affects both men and women, usually occurs after the age of 50. The following are some other risk factors for neuroendocrine tumours:
- A family history of multiple endocrine neoplasia type 1, a rare type of cancer syndrome, is inherited.
- Von Hippel-Lindau disease
- Tuberous sclerosis
How are these tumours diagnosed?
The diagnosis must be made by a medical professional. Your doctor will usually begin the diagnostic process with a fasting blood test. This test checks for elevated levels of somatostatin. A blood test is often followed by one or more diagnostic scans or X-rays:
- Endoscopic ultrasound
- Computed tomography
- Actrioscan (a radiological scan)
- Magnetic resonance
These tests allow your doctor to look at the tumour, which may or may not be cancerous. Most somatostatinoma is cancer. Surgery is the only way to determine if your tumor is cancerous.
How are they treated?
It is most often treated by surgical removal of the tumour. If the tumour is cancerous and cancer has spread (a condition called metastasis), surgery may not be an option. In the case of metastasis, your doctor will treat and monitor any symptoms that may cause somatostatinoma.
Associated conditions and complications
Some conditions may include the following:
- Van Hippel-Lindau syndrome
- Neurofibromatosis type 1
These are usually appear at a later stage. In the final stage, cancerous tumours are likely to already metastasize. After metastasis, treatment is limited because surgery is not usually an option.
The survival rate for somatostatinomas
Despite the rare nature of somatostatinomas, the outlook for a 5-year survival rate is good. When a somatostatinoma can be removed surgically, the survival rate is nearly 100 per cent within five years of removal. The five-year survival rate for those treated after the disease metastasis was 60 per cent.
The important thing is to get a diagnosis as soon as possible. If you have some symptoms, you should make an appointment with your doctor as soon as possible. Diagnostic tests can identify the specific cause of your symptoms.
If your doctor diagnoses you with this disease, the earlier you start treatment, the better your outlook.