Some tumors that arise from MEN 1 are malignant. People with MEN 1 usually develop a rare form of pancreatic cancer called islet cell carcinoma. Between 200 and 1,000 new cases are diagnosed in the United States each year. These tumors are usually soft and fleshy and develop slowly. Patients with islet cell carcinoma have a more favorable prognosis than people with other types of pancreatic cancer.
Symptoms
The two major types of islet cell carcinoma are gastrinoma and insulinoma. These are based on the type of hormone produced. Gastrinoma releases gastrin, which causes stomach or intestinal ulcers that can rupture the stomach or intestine. Insulinoma produces too much insulin, interfering with insulin secretion and sugar regulation. This causes hypoglycemia, or low blood sugar. Symptoms of insulinoma include restlessness, irritability, sweating and flushing. Tumors are often discovered after patients report these problems or symptoms to their doctor.
Diagnosis
An adenoma or carcinoma can be found using blood tests that measure the level of hormones or a CT scan that detects tumors. It is recommended that people who have inherited MEN 1 undergo these tests to determine which adenomas are at risk of becoming cancerous. Pancreatic malignancy is rare before age 30.
The blood tests measure the level of parathyroid hormone and calcium to detect hyperparathyroidism; prolactin to detect overactivity of the pituitary gland; and gastrin to detect overactivity of the pancreas.
Patients usually undergo a CT scan or ultrasound test to confirm the presence of an adenoma and whether it may develop into a malignant tumor. A CT scan (computed tomography) consists of an X-ray that takes pictures of the body from many different angles. Then, a computer will interpret all of these images into a detailed, cross-sectional view.
Staging
Islet cell carcinoma is not formally staged, but it is divided into three categories, depending on how far it has spread. These are:
1. Cancers occurring in one site
2. Cancers occurring in several sites
3. Cancer that has spread to lymph nodes near the original tumor or to distant sites in the body, such as the lungs or bones.
Treatment
Treatment of islet cell carcinoma is intended to treat the symptoms of the overactive glands. Most doctors prefer conservative treatment with drugs that control hormonally induced symptoms. Surgery is not advised unless it is necessary, because pancreatic procedures can be very risky. Surgery is used to remove tumors that are creating an obstruction, but have not spread beyond the pancreas. Tumors that cannot be surgically removed are treated with drugs that control hormonally induced symptoms. Omeprazole is effective in patients with gastrinoma and octreotide helps reduce hormone production.
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