Multiple endocrine neoplasia

This is a group of disorders presenting problems with the home-producing glands of the endocrine system. Hormones in the body act as chemical messengers that help regulate the function of cells and tissues throughout the body. Hormones are involved in the function of regulating heart rate, blood pressure and the body temperature. This disorder normally involves the tumors that develop in at least two endocrine glands which can either be cancerous or noncancerous. It can also develop in other organs and tissues of the body which can be life-threatening when the tumors become cancerous. The major forms of multiple endocrine neoplasia are type 1 and type 2 and which are based on the hormones involved and the symptoms that develop.

Multiple endocrine neoplasia type 1

This is the disorder presenting the noncancerous tumors that develop from various glands of the endocrine system. In this type of the disorder, the benign tumors often involve the parathyroid, pituitary glands and pancreas which results in the excessive amounts of hormones that are secreted into the bloodstream causing its associated symptoms. In some cases, the benign tumors can become cancerous. The symptoms are based on the affected glands which can become hyperactive. There are more than 20 different endocrine and non-endocrine tumors that are associated with this type of multiple endocrine neoplasia. The parathyroid gland is most commonly affected presenting benign growths developing by the age of 50. As the result, hyperparathyroidism causes too much calcium accumulation in the blood which can result in kidney stones and damage of the kidneys. The additional symptoms of hyperparathyroidism include ulcers, indigestion, high blood pressure, constipation, muscle and bone pain. Around 40% of the affected individuals develop multiple gastrinomas which are benign tumors that secrete gastrin. The high level of gastrin causes abdominal pain, esophageal reflex and peptic ulcers. In around 25% of the cases, benign growths of the pituitary gland occur and around 10-15% of individuals can develop slow-growing carcinoid tumors which can develop in the stomach, bronchial tubes and the thymus.

Multiple endocrine neoplasia type 2

There are two subtypes; type 2A and type 2B which can develop as the result of a spontaneous genetic mutation that can occur for no reason. The symptoms of this type can occur in infancy, early childhood, in adolescence or young adulthood. Most of the affected individuals develop the medullary thyroid carcinoma that can occur early in life. The three most common glands that become affected includes thyroid, parathyroid and the adrenal glands. Medullary thyroid carcinoma is the initial symptom that occurs that presents pain in the neck between 15 to 20 years of life. In around 50% of the cases, the benign tumor in the adrenal gland can develop. In around 20-30% of the affected individuals, hyperparathyroidism can occur as the result of the overgrowth of parathyroid tissue.

References

https://rarediseases.org/rare-diseases/multiple-endocrine-neoplasia-type/

http://www.cancer.net/cancer-types/multiple-endocrine-neoplasia-type-2

https://rarediseases.org/rare-diseases/multiple-endocrine-neoplasia-type-1/

http://www.cancer.net/cancer-types/multiple-endocrine-neoplasia-type-1

https://ghr.nlm.nih.gov/condition/multiple-endocrine-neoplasia#genes