• Multiple Endocrine Neoplasia

    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 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.

    Multiple Endocrine Neoplasia Treatment at Dana-Farber/Boston Children's

    Children with MEN1 and MEN2 are treated at Dana-Farber/Boston Children's 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.

    Continue reading for more information about MEN or visit the Endocrine-Oncology Program page to learn more about our expertise or meet our treatment team.

    How are multiple endocrine neoplasias classified?

    Multiple endocrine neoplasias are traditionally divided into two types, MEN1 and MEN2.

    • MEN type 1 usually involves tumors of the parathyroid glands, pituitary gland, and pancreas (usually pancreatic islet cell tumors).
    • MEN type 2 usually increases the risk of developing medullary thyroid cancer or pheochromocytomas. MEN2 is often further divided into types 2A and 2B. Type 2A is also associated with hyperparathyroidism (overactive parathyroid glands). Type 2B is often linked to growths in the body’s mucosal surfaces, known as neuromas, and very early development of medullary thyroid cancer.

    What are the causes and symptoms of multiple endocrine neoplasias?

    As a parent, you undoubtedly want to know what may have caused your child’s condition.

    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 symptoms of 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 may be suggested because of a family history.

    Some symptoms of MEN1 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, problems with fertility, or discharge of fluid from the nipples from pituitary gland tumors.

    Some symptoms of MEN2 might 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.

    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.

    How are multiple endocrine neoplasias diagnosed?

    The first step in treating your child is forming an accurate and complete diagnosis. MEN1 or MEN2 are 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 he or she develops tumors or other associated problems. Your child’s physician may order a number of different tests including:

    • A physical exam and complete medical history.
    • Genetic tests to determine genetic alterations in the MEN1 or RET gene. Genetic counselling to understand family history.
    • Blood and urine tests.
    • A biopsy, a tissue sample taken from the tumor. The tumor's appearance under a microscope helps doctors to make a diagnosis so the appropriate treatments can be recommended.
    • Magnetic resonance imaging (MRI), a diagnostic procedure that produces detailed images of the area where the tumor is located. An MRI uses a combination of large magnets, radiofrequencies, and a computer to analyze organs and structures within the body.
    • A computerized tomography scan (CT/CAT scan), an imaging technique that provides more detailed pictures than X-rays.
    • An ultrasound, particularly for thyroid tumors in patients with MEN2.
    • 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 treatments for multiple endocrine neoplasias?

    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, the use of high-energy rays from a specialized machine to damage or kill cancer cells and shrink tumors. This is often used together with surgery, either before or after removal of the tumor.
    • Chemotherapy, a drug treatment that aims to destroy or shrink cancer cells, may be given before or after surgery.
      • Different groups of chemotherapy drugs work in different ways. 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; 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.
    • Genetic counselling to discuss future reproductive options.

    What is the latest research on multiple endocrine neoplasias?

    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.

    Clinical Trials

    Clinical trials, or 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.

    It’s possible that your child will be eligible to participate in one of our clinical trials. 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.

    What is the long-term outlook for multiple endocrine neoplasias?

    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.

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