Treatment at Mayo Clinic

By Mayo Clinic Staff

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Mayo Clinic experts have a wealth of expertise in the latest interventions for primary multiple endocrine neoplasia, type 1 (MEN 1) tumors and those that have spread (metastasized). Treatment for MEN 1 depends on each person's situation and related conditions. In the early stages, specialists may recommend surgery to remove tumors as well as drug therapy to regulate hormones. If a tumor is cancerous and has spread, doctors may use surgery, radiation, chemotherapy and other treatments.

Below are examples of related conditions and treatment options:

  • Pituitary tumors. Doctors may recommend surgery or drug therapy based on the type of pituitary tumor. They will recommend regular follow-up screening.
  • Hyperparathyroidism. Most people with MEN 1 will develop too much calcium in the blood (hypercalcemia) and too much parathyroid hormone (hyperparathyroidism) by the time they reach age 50. Regular screening for people at risk can detect hyperparathyroidism much earlier. The most common treatment is surgery to remove three and a half of the four parathyroid glands (subtotal parathyroidectomy). If high blood-calcium levels return years later, doctors can use an ultrasound to find the tumor and partially destroy the remaining parathyroid tissue by injecting alcohol with a needle (ethanol ablation). Doctors may delay removing the parathyroid glands because their removal for MEN 1 rarely cures the condition.
  • Pancreatic and duodenal neuroendocrine tumors. People with MEN 1 frequently develop pancreatic and small intestine (duodenal) neuroendocrine tumors. These can become malignant, so early detection and intervention is important. Treatment depends on the type and stage of the tumor.
  • Hypoglycemic syndrome. Some people with MEN 1 develop tumors associated with too much insulin (insulinomas). Although the tumors are most often not cancerous (benign), no effective medical treatment exists, and they can cause life-threatening insulin imbalance. So doctors may recommend surgery, which usually involves removing part of the pancreas (distal pancreatectomy) and any remaining pancreatic tumors.
  • Other pancreatic endocrine tumors. People with MEN 1 who develop malignant islet cell tumors may require an aggressive approach, including surgical removal of part of the pancreas, nearby lymph nodes and any remaining tumors. A variety of treatments are available if the tumor has spread to other sites, such as the liver.
  • Zollinger-Ellison Syndrome. Many people with MEN 1 develop Zollinger-Ellison Syndrome (ZES), which involves tumors that result in overproduction of gastric acid (gastrinomas) leading to ulcers and diarrhea. Doctors may prescribe medication such as proton pump inhibitors (PPIs). However, extensive surgery may be necessary to slow the progress of the disease.
  • Advanced pancreatic disease. If your disease is more advanced — for example, if it spread to the liver — treatment may involve liver surgery and radiofrequency ablation or injecting strong chemotherapy drugs directly into the liver (chemoembolization). When surgery is not an option, doctors may use other forms of chemotherapy or hormone-based treatments.
  • Adrenal tumors. Some people with MEN 1 develop tumors in their adrenal glands. Most do not produce hormones and are not cancerous, so they can be observed. However, if the tumors produce hormones or they're large and thought to be cancerous, doctors recommend removing them.
  • Carcinoid tumors. Carcinoid tumors in people with MEN 1 can develop in the lungs, thymus gland and gastrointestinal (GI) tract. Surgeons remove these tumors when they haven't spread to other areas. Doctors may use chemotherapy, radiation therapy or hormone-based therapy for advanced cases.
Nov. 19, 2012