What Is Childhood Multiple Endocrine Neoplasia (MEN)?
Multiple endocrine neoplasia (MEN) is an inherited genetic condition that causes tumors to grow in several of the body's hormone-producing endocrine organs. MEN tumors can be benign (noncancerous) or malignant (cancerous).
- There are two forms of MEN syndromes:
- MEN type 1: MEN1 involves tumors of the parathyroid glands, pituitary gland, and pancreas.
- MEN type 2: MEN2 is commonly associated with medullary thyroid cancer and pheochromocytomas. MEN2 contains types 2A and 2B. Type 2A is associated with hyperparathyroidism (overactive parathyroid glands). Type 2B is linked to growths in the body's mucosal surfaces, known as neuromas, and very early development of medullary thyroid cancer.
We provide two programs for children with MEN1 and MEN2, including our Endocrine-Oncology Program and Boston Children's Hospital Thyroid Center. Once in our care, we build a team to treat your child that includes pediatric experts in the areas of oncology, endocrinology, genetics, and surgery.
Causes and Symptoms of Childhood Multiple Endocrine Neoplasia
MEN1 and MEN2 are inherited disorders, meaning that they are usually the result of an abnormal gene that families pass down. Occasionally, these conditions can arise from a new gene abnormality that develops for unknown reasons in a patient without any family history. In general, MEN1 results from abnormalities in the MEN1 gene, while MEN2 can arise from abnormalities in the RET gene.
The symptoms of MEN vary among children. Symptoms might mimic other, more common ailments. Sometimes your child may not experience symptoms, but your doctor may evaluate for it because of family history.
MEN1 symptoms may include:
- Elevated calcium levels leading to weakness, fatigue, bone pain, constipation, and kidney stones from overactive parathyroid glands.
- Abdominal pain, stomach ulcers, vomiting, diarrhea, and weight changes from pancreatic islet cell tumors.
- Headaches, weight changes, visual disturbances, fertility problems, or discharge of fluid from the nipples from pituitary gland tumors.
MEN2 symptoms may include:
- Elevated calcium levels leading to weakness, fatigue, bone pain, constipation, and kidney stones from overactive parathyroid glands.
- High blood pressure, headaches, or sweating from pheochromocytomas.
- Lump or swelling in the neck, difficulty breathing or swallowing, or hoarseness due to medullary thyroid cancer.
These symptoms can also point to other conditions. If you notice any of these symptoms, have your child evaluated by your doctor as soon as possible.
How We Diagnose Childhood Multiple Endocrine Neoplasia
If there is a family history of MEN, your doctor may screen your child before problems develop. A doctor may also suspect MEN1 or MEN2 if a child develops cancers known to occur with MEN.
We confirm the diagnosis of MEN with several diagnostic tests, including genetic testing, advanced imaging studies, biopsy, and blood and urine tests.
If your child needs any additional testing, your doctor will discuss their recommendations with you. Once we have an accurate and complete diagnosis, we will develop a treatment plan that meets your child's unique needs.
How We Treat Childhood Multiple Endocrine Neoplasia
Our doctors treat many children with endocrine tumors, giving us the experience to develop the most effective treatment plan possible.
Treatment for MEN1 and MEN2 includes:
- Screening: We screen for tumors related to your child's condition.
- Surgery: We use surgery to remove any existing tumors. We may also perform surgery to prevent medullary thyroid cancer from developing in patients with MEN2.
- Radiation therapy: Often, we'll use radiation therapy before or after surgery to damage or eliminate cancer cells and shrink tumors.
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Chemotherapy: We may also use chemotherapy to further destroy or shrink cancer cells.
Different groups of chemotherapy drugs work in different ways. Often, we will use a combination of chemotherapy drugs. Your child may receive chemotherapy:
- Orally, as a pill to swallow.
- Intramuscularly, as an injection into the muscle or fat tissue.
- Intravenously, as a direct injection into the bloodstream or IV.
- Intrathecally, as a direct injection into the spinal column through a needle.
While chemotherapy can be very effective in treating certain cancers, the treatment can't differentiate normal healthy cells from cancer cells. As a result, there can be adverse side effects during treatment. Being able to anticipate these side effects can help the care team, child, and family prepare for, and in some cases prevent these complications from occurring.
- Genetic counseling: We help your child and family cope with the stress of hereditary cancer diagnosis while discussing practical considerations such as future reproductive options.
We also provide children with continued, comprehensive care in our pediatric cancer survivorship programs. We conduct regular medical evaluations and provide patient and family education, support groups, and counseling services.