Multiple endocrine neoplasia (MEN) is an inherited genetic multiple endocrine neoplasia that causes tumors to grow in several of the body’s hormone-producing, endocrine organs. MEN syndromes are traditionally divided into two forms: type 1 and type 2.
MEN type 1 (MEN1) typically involves tumors of the parathyroid glands, pituitary gland, and pancreas. MEN type 2 (MEN2) is more commonly associated with medullary thyroid cancer and pheochromocytomas. These tumors can be benign or malignant.
His family has a history of multiple endocrine neoplasia, a group of rare disorders that can make them more likely to develop tumors in the endocrine glands, including the thyroid.
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:
Some symptoms of MEN2 might include:
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.
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.
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:
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.
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:
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.
Children with MEN who had been treated for a tumor should visit a survivorship clinic yearly. Through the David 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.
Children with MEN1 and MEN2 are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Endocrine-Oncology Program and the Boston Children’s Hospital Thyroid Program. Our integrated pediatric oncology service offers — in one specialized program — the combined expertise of a leading cancer center and a premier children’s hospital. We build a team to treat your child consisting of oncologists, endocrinologists, genetic counselors, and surgeons.
Children who are treated through our Endocrine-Oncology Program benefit from the work of our basic and clinical researchers, who are striving to understand the scientific causes of endocrine cancers. Their work can result in the introduction of new treatment options. We are a world leader in translational research, bringing laboratory advances to the bedside and into doctors’ offices as quickly as possible.
Research studies evaluating new treatment approaches are a major offering at Dana-Farber/Boston Children’s. For many children with rare or hard-to-treat conditions, clinical trials provide new options.
In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG). If your child has a progressive or recurrent tumor, she may be eligible for a number of experimental therapies available through these groups or from one of our independent clinical investigators.
