How are MEN 2A and MEN 2B treated?

Treatment of MEN 2A and MEN 2 B focuses on surgical removal of tumors that might spread to other parts of the body or cause life-threatening biochemical disturbances. With either syndrome, it may be necessary to perform operations in stages to minimize the overall risk of complications. If pheochromocytomas are present, they should be removed in a separate operation before proceeding with any other operations. The general concern is that a pheochromocytoma may produce large amounts of biochemicals that could increase the blood pressure to dangerous levels when a patient is placed under general anesthesia.

Confirmed medullary thyroid cancer should be treated with surgical removal of the entire thyroid gland, followed by careful exploration and dissection of the lymph nodes in the neck. In most cases, it is recommended that the lymph node dissection should be performed in the central portion of the neck. If there is any concern that a tumor has grown to the point where it is likely to have spread to other lymph nodes, dissection is usually extended up along both sides of the neck. In the process of dissection, the surgeon examines and removes a number of lymph nodes. These are later examined by a pathologist to see if they contain medullary thyroid cancer cells.

Medullary thyroid cancer cells produce a biochemical substance called calcitonin that can be measured in blood samples. They may also produce a biochemical substance called carcinoembryonic antigen (abbreviated CEA) that can be measured in a similar way. Calcitonin and CEA levels can be measured before and after surgery to see if they drop significantly. They can also be followed over time to see if they start to rise again, indicating that medullary thyroid cancer may be recurring and further treatment may be necessary.

In cases of MEN 2A with suspected parathyroid hyperplasia, surgical exploration of the parathyroid glands is usually performed at the time that the thyroid gland is removed. If the parathyroids are noted to be enlarged, a surgeon may elect to remove most of the parathyroid tissue, taking a small fraction of it and transplanting it into the arm where it will continue to function to maintain the balance of calcium in the body.

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