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    Multiple Endocrine Neoplasia

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    Divya Ranasaria
    There are three main types of multiple endocrine neoplasia (MEN): MEN1, MEN2a, and MEN2b. MEN1 is characterized by tumors of the parathyroid glands, pancreas, and pituitary glands. MEN2a involves medullary thyroid cancer, pheochromocytoma, and parathyroid tumors. MEN2b also includes medullary thyroid cancer and pheochromocytoma, along with neuromas. Each type of MEN is caused by a specific genetic mutation and tends to run in families. While there is no cure, treatment focuses on surgically removing tumors when possible.

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    Multiple Endocrine Neoplasia

    Uploaded by

    Divya Ranasaria
    121 views4 pages
    There are three main types of multiple endocrine neoplasia (MEN): MEN1, MEN2a, and MEN2b. MEN1 is characterized by tumors of the parathyroid glands, pancreas, and pituitary glands. MEN2a involves medullary thyroid cancer, pheochromocytoma, and parathyroid tumors. MEN2b also includes medullary thyroid cancer and pheochromocytoma, along with neuromas. Each type of MEN is caused by a specific genetic mutation and tends to run in families. While there is no cure, treatment focuses on surgically removing tumors when possible.

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    There are three main types of multiple endocrine neoplasia (MEN): MEN1, MEN2a, and MEN2b. MEN1 is characterized by tumors of the parathyroid glands, pancreas, and pituitary glands. MEN2a involves medullary thyroid cancer, pheochromocytoma, and parathyroid tumors. MEN2b also includes medullary thyroid cancer and pheochromocytoma, along with neuromas. Each type of MEN is caused by a specific genetic mutation and tends to run in families. While there is no cure, treatment focuses on surgically removing tumors when possible.

    Copyright:

    © All Rights Reserved
    Download as DOCX, PDF, TXT or read online from Scribd
    Download as docx, pdf, or txt
    121 views4 pages

    Multiple Endocrine Neoplasia

    Uploaded by

    Divya Ranasaria
    There are three main types of multiple endocrine neoplasia (MEN): MEN1, MEN2a, and MEN2b. MEN1 is characterized by tumors of the parathyroid glands, pancreas, and pituitary glands. MEN2a involves medullary thyroid cancer, pheochromocytoma, and parathyroid tumors. MEN2b also includes medullary thyroid cancer and pheochromocytoma, along with neuromas. Each type of MEN is caused by a specific genetic mutation and tends to run in families. While there is no cure, treatment focuses on surgically removing tumors when possible.

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    © All Rights Reserved
    Download as DOCX, PDF, TXT or read online from Scribd
    Download as docx, pdf, or txt
    of 4

    Multiple Endocrine Neoplasia(MEN)

    These are conditions that cause overactivity and enlargement in certain endocrine glands. MEN
    syndromes are usually (but not always) inherited conditions and therefore they run in families. When
    they are inherited, they are passed from one generation to the next.

    There are three main types of multiple endocrine neoplasia (MEN) - MEN 1, MEN 2a and MEN2b.

    MEN 1 = Parathyroid tumors, pancreatic tumors, and pituitary tumors.

    MEN 2a = Medullary thyroid cancers (MTC), pheochromocytoma, and parathyroid tumors.

    MEN 2b = Medullary thyroid cancers, pheochromocytoma and neuromas.

    There are specific genetic causes for each of the three types of MEN. Any particular MEN family will have
    only one type of MEN, either MEN 1, MEN 2a or MEN 2b.

    MEN1(Wermer’s MEN 2 MEN2B MEN X


    syndrome) (MEN2A Or Sipple
    syndrome)
    Compon  Parathyro  Medullary  Medullary  Hyperparathyr
    ents id carcinoma carcinoma oidism
    hyperplas thyroid thyroid  Pituitary
    ia or  Pheochromoc  Pheochromoc adenoma
    adenoma ytoma ytoma  Pancreatic
     Pancreati  Parathyroid  Megacolon NET
    c hyperplasia or  Marfanoid  Gonadal,
    neuroend adenoma features adrenal, renal
    ocrine  Hirschsprung  Mucosal & thyroid
    tumor disease neuromas tumors
     Pituitary
    adenoma
     Bronchial
    and
    thymic
    carcinoid
    s
     Collageno
    mas
     Subcutan
    eous
    lipoma

    Gene/De MEN1 gene RET RET oncogene CDNKIB gene


    fect oncogene(cysteine (tyrosine kinase
    codon) domain)
    Chromos 11 10 10 12
    ome
    Transmis AD AD AD AD
    sion

    MEN1 (Wermer’s Syndrome)


    When parathyroid gland is involved:

     MC endocrine abnormality
     Hyperparathyroidism is MC manifestation(cardinal sign is parathyroid adenoma)
     Hypercalcemia is 1st biochemical abnormality detected in MEN1

    When pancreas is involved(Pancreatic neuroendocrine tumors)

     It is the 2nd MC manifestation


     Secrete pancreatic polypeptide which is non functioning type
     Functioning type in MEN 1 is gastrinoma followed by insulinoma
     MC increased pancreatic hormone: Pancreatic polypeptide>Gastrin>Insulin(PGI)

    Some people with type 1 disease develop pituitary gland tumors. Some of these tumors produce the
    hormone prolactin, causing menstrual abnormalities and often breast secretions (galactorrhea) in
    women who are not nursing and decreased sexual desire and erectile dysfunction (impotence) in men.

    In some people with type 1 disease, tumors or excessive growth and activity of the thyroid and adrenal
    glands develop. A small percentage of people develop a different type of tumor, known as carcinoid
    tumors.

    Diagnosis
     Genetic tests
     Hormone levels in blood and urine
     Imaging tests

    Tests are available to identify the genetic abnormality present in each of the multiple endocrine
    neoplasia syndromes. Doctors usually do these genetic tests in people who have one of the tumors
    typical of multiple endocrine neoplasia and in family members of people already diagnosed with one of
    the syndromes. Screening of family members, sometimes even before birth, is particularly important
    because about half of the children of people with a multiple endocrine neoplasia syndrome inherit the
    disease.

    Blood and urine tests are done to detect elevated hormone levels.

    Imaging tests, such as ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI),
    and positron emission tomography (PET), are also needed to help doctors determine locations of the
    tumors.

    Treatment

    Tumor removal
    Often removal of the thyroid gland

    Drugs

    No cure is known for any of the multiple endocrine neoplasia syndromes. Doctors treat the changes in
    each gland individually.

    A tumor is treated by removing it surgically when possible. Some small pancreatic islet cell tumors are
    not removed immediately but are monitored to see whether they are increasing and treated if they
    become large enough to cause problems.

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