Multiple endocrine neoplasias are the noncancerous and cancerous disease of endocrine glands. This disease is an inherited disease caused by mutations in the genes passed on from parents to their children. It runs in families. It affects pituitary, parathyroid, thyroid, adrenal glands, and pancreas. It may involve more than one endocrine gland at a time. It affects the production of hormones in these glands. Due to the mutated genes, the affected endocrine gland may become overactive. It results in various symptoms in the body due to impaired hormone secretion. It is an incurable disease and cannot be prevented. Genetic screening tests may detect this condition.
Is Multiple Endocrine Neoplasia Cancer?
All multiple endocrine neoplasias are not cancerous. Multiple Endocrine Neoplasia (MEN) 1 type is a benign tumor (non-cancerous) that is not likely to develop into a malignant tumor (cancerous). One out of three tumors of islet cells of the pancreas can develop the cancerous state. MEN 2A and MEN 2B type can lead to cancer in thyroid gland in 98% of cases. This cancer is known as medullary thyroid cancer. Both of them can also cause pheochromocytoma, mucosal neuroma on the tongue, lips and inside the entire gastrointestinal tract. These are benign tumors. MEN 2B may also lead to parathyroid tumors of benign nature. (1)
This syndrome develops either in infant age or adult age. It affects males and females equally. It runs in families. Those patients who have a family history are more at risk to suffer from this syndrome.
Multiple endocrine neoplasias are the inherited disease of overactive endocrine glands that run in families. It was also called as Wermer syndrome in the past. It is caused by the transfer of mutated or defective genes from parents to children. The defective gene causes overproduction of hormones by one or more endocrine glands in the affected person at the same time. It may involve endocrine glands like a parathyroid gland, pancreas, pituitary, thyroid and adrenal gland. (2)
Cancer is a condition characterized by uncontrolled growth and multiplication of cells leading to the formation of tumors in the affected areas. These tumors are of two types, benign and malignant. Benign tumors are non-cancerous tumors marked by non-regulated multiplication of cells. These tumors are not fatal and it may compress the surrounding tissues and organs. They are slow growing tumors. They also spread to other parts of the body.
Malignant tumors are a cancerous overgrowth of the cells in the affected areas in an uncontrolled manner. These tumors can spread to other parts of the body. This process is called metastasis. They are rapidly growing tumors and adversely affect the health of the individual. They are serious forms of cancer that can be life-threatening if not treated well.
Multiple Endocrine Neoplasia Types
There are three types of multiple endocrine neoplasia syndromes-
- MEN 1- it develop tumors in the pituitary gland, parathyroid gland, and pancreas. It usually develops in childhood or adulthood. (4)
- MEN 2A- Multiple endocrine neoplasias 2A affect the thyroid gland, adrenal gland and parathyroid gland resulting in the formation of tumors. It usually starts at the age of 30 years. (3)
- MEN 2B- Multiple endocrine neoplasia 2B influences the activity of thyroid gland and adrenal gland. It may appear in childhood before the age of 10 years.
Multiple endocrine neoplasia syndromes are induced by mutations in the genes transferred from parents to their children. The mutation in certain genes is the most common cause of this disorder. These defective genes are MEN 1, RET, and CDKN1B genes. These genes carry information for functioning growth, and multiplication of endocrine glands.
Conclusion
Multiple endocrine neoplasias are rare disorders of endocrine glands marked by the appearance of tumors in them. These tumors can be benign or malignant. Only a few cases of multiple endocrine neoplasias develop a malignancy. About 90-98 % of multiple endocrine neoplasia 2A and 2B may show such cancerous growths usually in thyroid glands.
