![Multiple Endocrine Neoplasia Syndromes in Children](https://images.medicinenet.com/images/article/main_image/human-glands-lobes-thyroid-parathyroid-illustration-rendering.jpg?output-quality=75)
Multiple endocrine neoplasia (MEN) syndromes in children are genetically inherited disorders. They cause tumors that grow into several hormone-producing glands (endocrine glands). Childhood MEN involves at least two hormone-producing glands. These tumors can be either benign (cancerous) or malignant (noncancerous).
There are two major types of MEN:
- MEN I: One or more endocrine glands of your child could be involved. MEN1 commonly involves pituitary glands, parathyroid glands, and pancreas.
- MEN II: Rare type of MEN in children and commonly involves the thyroid (especially the medullary thyroid gland), parathyroid, and adrenal glands. MEN2 has two subtypes:
- MEN IIA: Mostly associated with an increased risk of medullary thyroid cancer, pheochromocytoma, and primary hyperparathyroidism.
- MEN IIB: Mostly associated with an increased risk of medullary thyroid cancer and pheochromocytoma and mucosal neuromas (a benign growth on lips and tongue).
MEN I occurs in 1 out of 30,000 individuals, and MEN IIA occurs in 1 out of 35000 and is the most common. MEN IIB accounts for only five percent of all MEN II cases.
What Are the Symptoms of MEN?
Symptoms of multiple endocrine neoplasia I
- Increased calcium levels
- Kidney stones from the hyperactive parathyroid gland
- Abdominal pain
- Vomiting
- Weight changes
- Weakness
- Fatigue
- Bone pain
- Constipation
- Headache
- Vision problems
Symptoms of multiple endocrine neoplasia II
- Increased calcium levels
- Kidney stones from the hyperactive parathyroid gland
- Increased blood pressure
- Excessive sweating
- Fatigue
- Bone pain
- Constipation
- Lump in the neck, making breathing and swallowing difficult
- Hoarse voice due to medullary thyroid cancer
What Are the Causes of MEN?
Multiple endocrine neoplasia (MEN) I and II are inherited disorders. The faulty gene that runs in the families causes these. Sometimes, new gene defects raise without any known reason. Usually, a defect in the MEN1 gene causes MEN I.
- The MEN1 gene helps produce menin (a protein).
- Menin functions as a tumor suppressor and prevents abnormal, the rapid growth of cells, but any abnormality or defects in the gene may result in MEN I.
A defect in the RET gene causes MEN II.
How is MEN Diagnosed?
Gene testing is the most effective method to diagnose multiple endocrine neoplasia (MEN).
The following methods are used alone or in combination to detect MEN:
- Family history
- General and physical examination
- Blood tests
- Ultrasound of thyroid
- CT scan
- Biopsy
- Nuclear medicine studies
How to Treat MEN
There is currently no cure for any of the multiple endocrine neoplasia (MEN) syndromes. Doctors treat each gland’s changes separately.
Once diagnosed with MEN, proper care and follow-ups must be done with doctors. The type of treatment depends on the child's age and the type of mutation that has occurred.
- Especially in MEN II, children have a high risk of thyroid cancer, and prophylactic thyroidectomy is recommended
- Radiation therapy
- Chemotherapy
Early diagnosis and treating the condition may prevent mortality related to the disease.
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Multiple endocrine neoplasia. https://medlineplus.gov/genetics/condition/multiple-endocrine-neoplasia/#causes
Childhood Multiple Endocrine Neoplasia (MEN) Syndromes Treatment (PDQ®)–Patient Version. https://www.cancer.gov/types/multiple-endocrine-neoplasia/patient-child-men-syndromes-treatment-pdq
Childhood Multiple Endocrine Neoplasia. https://www.dana-farber.org/childhood-multiple-endocrine-neoplasia/