What is Somatostatinoma?
Somatostatinoma is an extremely rare medical condition in which there is development of malignant tumors in and around pancreas which secretes the hormone somatostatin. Somatostatinoma almost all the times begin in the pancreas, although pancreas is not the only organ where these tumors develop. Somatostatinoma although being a malignant tumor is still curable if diagnosed early and removed in its entirety before it has a chance to spread to other areas of the body.
As stated, Somatostatinoma is a very rare condition and is estimated to occur in one out of a million people in the United States. Somatostatinoma are usually diagnosed in individuals who are in the age range of above 60 years. This is partly because of the slow growing nature of the tumor. Both males and females can equally be affected by Somatostatinoma. There are certain medical conditions like Multiple Endocrine Neoplasia type 1 or MEN-1 syndrome and neurofibromatosis which begin much earlier in life and may predispose an individual to Somatostatinoma.
What Causes Somatostatinoma?
Somatostatinoma is basically caused due to uncontrolled growth of cells that produce the hormone somatostatin. These cells are found mainly in the pancreas and the duodenum. The reason as to why the growth of these cells becomes abnormal is not yet clearly known but certain medical conditions like MEN-1 syndrome and neurofibromatosis contribute in development of Somatostatinoma. Pheochromocytoma is yet another medical condition which can be held responsible for development of Somatostatinoma.
What are the Symptoms of Somatostatinoma?
Some of the symptoms of Somatostatinoma are
- Abnormally elevated blood sugar levels which can cause diabetic complications like ketoacidosis
- Presence of gallstones which may not be evident if the stones are small but larger and increased quantity of stones may cause inflammation of the gallbladder.
- Steatorrhea or fatty stools is yet another symptom of Somatostatinoma in which the stool will have a foul odor and have a greasy appearance
- Persistent diarrhea as a result of impaired digestion due to Somatostatinoma
- Unintentional weight loss, especially more marked if the tumor growth is in the pancreas
- Bowel obstruction may be experienced as a result of Somatostatinoma with tumors developing in the duodenum.
How is Somatostatinoma Diagnosed?
As Somatostatinoma is a very slow growing tumor hence most of the times the diagnosis of this condition is significantly delayed and by the time Somatostatinoma is diagnosed it is already spread to other parts of the body. However, there are certain diagnostic factors through which one may be able to diagnose Somatostatinoma early. If an individual has predisposing factors for Somatostatinoma like MEN-2 syndrome or neurofibromatosis then Somatostatinoma can be suspected if the individual has symptoms like those mentioned above.
In case a Somatostatinoma is suspected to be the cause of the symptoms then it can be confirmed with a blood draw which will show abnormally high levels of somatostatin hormone due to the presence of a tumor. Once the laboratory studies confirm presence of high levels of somatostatin then further investigations will be carried out in the form of radiological studies like CT and MRI scan to identify the exact location of the tumor as to whether they are in the pancreas or in the duodenum.
These tests will also help the physician check whether the tumor is still localized or has it spread to include other parts of the body. This is called staging of the tumor. Once these tests are done and the result turns out to be positive then a diagnosis of Somatostatinoma is confirmed.
How is Somatostatinoma Treated?
The best way to treat Somatostatinoma is to excise the tumor in its entirety and then following it up with chemotherapy. Chemotherapy is reserved for patients in which the Somatostatinoma has spread to other parts of the body. If the tumor is identified early or has not spread then surgical excision of the tumor is in majority of the cases curative and the patient is completely free of Somatostatinoma.
In cases where the tumor is located at a place where it cannot be resected then only chemotherapy is done to kill the cancer cells and prevent any further progression of the tumor. It should be mentioned here that chemotherapy as a treatment is not curative and is done only when the tumor is inaccessible for surgical removal of Somatostatinoma.