Multiple endocrine neoplasia (MEN) is a hereditary condition, where an individual may have developed tumours in one or more endocrine glands. Endocrine glands are the glands that secrete hormone into the blood directly rather than secreting into a duct. Tumours can be benign (noncancerous) or malignant (cancerous). The glands that are commonly affected include pituitary, thyroid, parathyroid, adrenal, and pancreas.
The major types of MEN syndromes are MEN type I and MEN type II.
Multiple endocrine neoplasia type I – It is caused by a defect in the gene coding for the protein menin, which is located on chromosome 11. The tumour commonly affects the parathyroid, pancreas, and pituitary glands. The common symptoms include abdominal pain, black stools, headache, burning sensation in upper abdomen, loss of appetite, weakness, loss of muscle coordination, and nausea and vomiting. The tumour can be diagnosed by tests such as abdominal CT and MRI scan, blood tests, and parathyroid biopsy.
- If the tumour has affected the pituitary gland then anti-cancer drugs are given to prevent the tumour growth and to reduce the prolactin level. Prolactin is a peptide hormone produced by the pituitary gland.
- If the tumour has affected the parathyroid gland then the entire gland may be removed. Parathyroid gland controls the calcium production and if these glands are removed the body will not be able to produce calcium which may lead to formation of kidney stones, thinning of bone, and high blood pressure. Hence the total removal of the gland is not done initially. If the entire gland is removed during the surgery, your doctor may suggest for hormone replacement therapy.
- Your doctor may prescribe certain medicines to lower the production of gastric acid produced by some tumours and to reduce the risk of ulcers.
Multiple Endocrine neoplasia (MEN) type II – It is caused by a defect in the RET gene. It commonly causes medullary carcinoma in the thyroid gland. Pheochromocytoma (adrenal gland tumour) may be seen in nearly half of the patients and some patients may also have hyperplasia of parathyroid gland.
Multiple endocrine neoplasia type II is subdivided into three types, MEN IIa, MEN IIb, and familial medullary thyroid carcinoma (FMTC). The MEN type IIa is most commonly occurring subtype where medullary carcinoma occurs in early stages of adulthood, some of the individuals develop pheochromocytoma and some develop hyperparathyroidism. In MEN type IIb the medullary carcinoma occurs in the early childhood and some individuals develop pheochromocytoma but hyperparathyroidism may not be observed. In FMTC, medullary carcinoma occurs in several members of the same family without the occurrence of pheochromocytoma and hyperparathyroidism.
The most common symptoms include tumour pressing on the nearby structures, breathing problems, cough, diarrhoea, abdominal pain, chest pain, nervousness, palpitations, irritability and weight loss. Type II MEN disorder can be diagnosed by physical examination and diagnostic tests such as abdominal CT and MRI scan, thyroid scan, ultrasound scan, and biopsies of the glands.
Surgery is done to remove the tumour in the adrenal gland. Medullary carcinoma is treated by removal of the thyroid gland and the surrounding lymph nodes. Your doctor may suggest hormone replacement therapy if the entire thyroid gland is removed. Early diagnosis and surgery can cure the disease condition. If the child is a carrier of RET gene, then thyroid gland is surgically removed before it becomes cancerous.
The purpose of hormone replacement therapy is to compensate for the thyroid hormones that are lost when the thyroid glands are removed because of which the body will no longer be able to produce thyroid hormones, leading to hypothyroidism. Thyroid hormones help to control body metabolism, when the hormone level is reduced the metabolism rate will be impaired. Hormone replacement therapy can address the issue of hypothyroidism.
Thyroid cancer is an abnormal growth of the cells of the thyroid gland, a butterfly-shaped gland located in the front of your neck just below the voice box (larynx). Thyroid gland secretes hormones that help regulate the body’s metabolism and levels of calcium. Thyroid cancer is more common in women than men. People who are exposed to high levels of radiation to the neck and have a family history of thyroid cancer and goitre (enlargement of thyroid gland) are at a higher risk of developing thyroid cancer.
Generally, surgery involving thyroid gland removal is the most common treatment of thyroid cancer. Total thyroidectomy is a surgical procedure to remove all of the thyroid gland. Subtotal or partial thyroidectomy is a surgery to remove part of the thyroid gland. Your doctor may also remove the lymph nodes if the cancer has spread to the lymph nodes. The surgery is performed under general anaesthesia. The surgeon removes the thyroid gland by making a 3-inch to 4-inch incision in the middle of your neck, on top of the thyroid gland. A small tube (catheter) will be placed into the area to drain the accumulated blood and fluids.
Thyroidectomy carries a risk of bleeding and infection, damage to your parathyroid glands resulting in low calcium levels in your blood. There is also a risk of damage to the nerves connected to your vocal cords and larynx, which can cause, hoarseness, coughing, swallowing problems, or speaking problems.