MEN-2 (Multiple Endocrine Neoplasia-2) actually covers a number of related conditions. It is commonly divided into MEN-2a and MEN-2b. Both conditions are usually inherited, with a 50:50 risk of occurring in the child of a parent with the disease. They may also occur in people without other affected family members, because of a problem with the gene that causes MEN-2 occurring after birth (known as an index case). MEN-2a occurs in approximately 1 in 100,000 people, but MEN-2b is much less common.
MEN-2a produces problems in 3 endocrine glands:
- Medullary Thyroid Cancer (MTC). This occurs in 90% of people with MEN-2a. The age of cancer development varies between different families. Surgery to remove the thyroid gland in children of people with MEN-2a before they develop cancer is now well established, and drastically reduces the risk of subsequent cancer development.
- Phaeochromocytoma. This occurs in 50% of people with MEN-2a. It is a tumour that is usually benign, and occurs in the adrenal glands. These tumours produce high levels of the chemicals adrenaline and noradrenaline that may lead to high blood pressure, strokes and heart problems. Treatment is by removal of the affected adrenal gland, which should be performed by minimally invasive (laparoscopic) surgery.
- Hyperparathyroidism. This is overactivity of the parathyroid glands, and occurs in 20-30% of people with MEN-2a. Treatment is by surgical removal of the overactive glands.
People with MEN-2b may be affected by medullary thyroid cancer and phaeochromocytoma, but hyperparathyroidism is not usually a problem. They may, however, have problems with the nerve cells in the bowel.
In addition to MEN-2a and 2b, some people have inherited thyroid cancer of the medullary type without problems of other endocrine glands (known as Familial Medullary Thyroid Cancer (FMTC)).
Because of the complex nature of all of the above conditions, all people with these conditions should be seen in a clinic run by specialist teams that can monitor all of these issues, and offer treatment when problems arise.
At University Hospital Aintree a specialist MEN clinic is provided. In addition to Mr Hardy, this clinic is run by doctors that specialise in endocrinology, medical genetics and paediatric endocrinology, allowing a multidisciplinary approach to monitoring and treatment.