Mayo Clinic experts have a wealth of expertise in the latest interventions for the tumors associated with multiple endocrine neoplasia, type 1 (MEN 1) if they are in one area (localized) or if they 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 treatments such as radiofrequency ablation or chemoembolization.
Below are examples of related conditions and treatment options:
Sept. 17, 2014
- 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) due to too much parathyroid hormone (hyperparathyroidism) by the time they reach age 50. This can lead to the development of kidney stones, osteoporosis and other symptoms. Regular screening for people at risk can detect hyperparathyroidism before complications occur.
The most common treatment is surgery to remove three and a half of the four parathyroid glands (subtotal parathyroidectomy). The half gland that's left can maintain normal levels of calcium. However, with time, the remaining parathyroid tissue usually starts to overproduce parathyroid hormone again.
When re-treatment is required, options include a second surgery, transplantation of parathyroid tissue to the arm or chest wall, or alcohol injection into a portion of the parathyroid gland under ultrasound guidance.
- Neuroendocrine tumors. People with MEN 1 frequently develop neuroendocrine tumors, also called islet cell tumors. These are usually located in the pancreas or small intestine (duodenum). These can become cancerous (malignant), so early detection and intervention is important. Some neuroendocrine tumors overproduce a normal pancreatic hormone, which results in symptoms related to high levels of the specific hormone. Treatment depends on the type and stage of the tumor.
- Hypoglycemic syndrome. Some people with MEN 1 develop tumors that produce too much insulin (insulinomas). Although the tumors are most often not cancerous, they can cause severely low levels of glucose, which can be life-threatening. More than one tumor may be present and doctors usually recommend surgery. In removing the tumor or tumors, often a portion of the normal pancreas needs to be removed (distal pancreatectomy).
- Zollinger-Ellison syndrome (ZES). Many people with MEN 1 develop 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.
- Metastatic neuroendocrine tumors. If your disease is more advanced — for example, if it has spread to the lymph nodes or liver — treatment may involve liver surgery, radiofrequency ablation or injection of 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 tumors do not produce hormones and are not cancerous, so they can be observed and not treated. However, if the tumors produce hormones or they're large and thought to be cancerous, doctors recommend removing them, usually with laparoscopic surgery.
- 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.
- Melmed S, et al. Williams Textbook of Endocrinology. 12th ed. Philadelphia, Pa.: Saunders Elsevier; 2011. https://www.clinicalkey.com. Accessed July 9, 2014.
- Multiple endocrine neoplasia type 1. National Institute of Diabetes and Digestive and Kidney Diseases. http://endocrine.niddk.nih.gov/pubs/men1/men1.aspx. Accessed July 12, 2014.
- Riggin EA. Decision Support System. Mayo Clinic, Rochester, Minn. June 12, 2014.
- Thakker RV. Multiple endocrine neoplasia. Medicine. 2013;41:562.
- Walls GV. Multiple endocrine neoplasia (MEN) syndromes. Seminars in Pediatric Surgery. 2014;23:96.
- Multiple endocrine neoplasia, type 1 (MEN 1). The Merck Manual for Health Care Professionals. http://www.merckmanuals.com/professional/endocrine_and_metabolic_disorders/multiple_endocrine_neoplasia_men_syndromes/multiple_endocrine_neoplasia_type_1_men_1.html. Accessed July 13, 2014.
- Nippoldt TB (expert opinion). Mayo Clinic, Rochester, Minn. July 19, 2014.
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