Multiple Endocrine Neoplasia | Symptoms & Causes
What are the symptoms of multiple endocrine neoplasia?
The symptoms of multiple endocrine neoplasia (MEN) may vary from child to child and depend on the type of the disease. Symptoms might mimic other, more common ailments. Sometimes your child may not experience symptoms but MEN type 1 or 2 may be suggested because of a family history.
Some symptoms of MEN1 may include:
- elevated calcium levels
- abdominal pain, vomiting and weight changes due to pancreatic islet cell tumors
- headaches, visual disturbances and problems with fertility from pituitary gland tumors
Some symptoms of MEN2 might include:
- elevated calcium levels
- kidney stones from overactive parathyroid glands.
- high blood pressure, headaches or sweating
- lump or swelling in the neck
- difficulty breathing or swallowing
Because many of these symptoms can also point to other conditions, it’s important to have your child evaluated by a qualified medical professional right away.
What causes MEN?
Both MEN1 and MEN2 are inherited disorders, meaning that they are usually the result of an abnormal gene that is passed down in families. Occasionally the conditions can arise from a new gene abnormality that develops for unknown reasons in a patient without a family history of these conditions. In general, MEN1 results from abnormalities in the MEN1 gene, while MEN2 can arise from abnormalities in the RET gene.
Multiple Endocrine Neoplasia | Diagnosis & Treatments
How is multiple endocrine neoplasia diagnosed?
The first step in treating your child is forming an accurate and complete diagnosis. Multiple endocrine neoplasia (MEN) is sometimes diagnosed if your child has developed cancers known to occur with MEN. If there is a family history of these conditions, your child may be tested for them before they develop tumors or other associated problems. Your child’s physician may order a number of different tests including:
- physical exam and complete medical history
- genetic tests to determine genetic alterations in the MEN1 or RET gene
- blood and urine tests.
- biopsy
- magnetic resonance imaging (MRI)
- computerized tomography scan (CT/CAT scan)
- ultrasound
- nuclear medicine studies
There may be other diagnostic tests that your doctor will discuss with you depending on your child's individual situation. After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then, we will meet with you and your family to discuss the results and outline the best possible treatment options.
What are the treatment options for multiple endocrine neoplasia?
Treatment for your child's MEN1 or MEN2 depends on the type of condition and whether or not tumors have developed. Your child’s doctor may recommend:
- screening for tumors related to your child’s condition
- surgery to prevent the development of medullary thyroid cancer in patients with MEN2
- surgery to remove any tumors that have developed
- radiation
- chemotherapy
- genetic counselling to discuss reproductive options
Different groups of chemotherapy drugs work in different ways. Your child may receive chemotherapy orally, intramuscularly, intravenously or intrathecally as a direct injection into the spinal column through a needle. Often, a combination of chemotherapy drugs is used.
While chemotherapy can be quite effective in treating certain cancers, the drugs cannot 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 and, in some cases, prevent these complications from occurring, if at all possible.
What is the long-term outlook for children with multiple endocrine neoplasia?
Children with MEN who had been treated for a tumor should visit a survivorship clinic yearly. Through the& B. Perini, Jr. Quality of Life Clinic, our cancer survivorship clinic, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team. In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists, or alternative/complementary therapy specialists. We also offer patient and family education, psychosocial assessment, genetic counseling, reproductive counseling, and opportunities to speak with other childhood cancer survivors.