Multiple Endocrine Neoplasia

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R.v. Thakker - One of the best experts on this subject based on the ideXlab platform.

  • Multiple Endocrine Neoplasia type 1
    Oxford Textbook of Endocrinology and Diabetes, 2011
    Co-Authors: R.v. Thakker
    Abstract:

    Multiple Endocrine Neoplasia (1, 2) is characterized by the occurrence of tumours involving two or more Endocrine glands within a single patient. The disorder has previously been referred to as Multiple Endocrine adenopathy (MEA) or the pluriglandular syndrome. However, glandular hyperplasia and malignancy may also occur in some patients and the term Multiple Endocrine Neoplasia (MEN) is now preferred. There are two major forms of Multiple Endocrine Neoplasia, referred to as type 1 and type 2, and each form is characterized by the development of tumours within specific Endocrine glands (Table 6.11.1). Thus, the combined occurrence of tumours of the parathyroid glands, the pancreatic islet cells, and the anterior pituitary is characteristic of Multiple Endocrine Neoplasia type 1 (MEN 1), which is also referred to as Wermer’s syndrome. However, in Multiple Endocrine Neoplasia type 2 (MEN 2), which is also called Sipple’s syndrome, medullary thyroid carcinoma (MTC) occurs in association with phaeochromocytoma, and three clinical variants, referred to as MEN 2a, MEN 2b and MTC-only, are recognized (Table 6.11.1). Although MEN 1 and MEN 2 usually occur as distinct and separate syndromes as outlined above, some patients occasionally may develop tumours that are associated with both MEN 1 and MEN 2. For example, patients suffering from islet cell tumours of the pancreas and phaeochromocytomas or from acromegaly and phaeochromocytoma have been described, and these patients may represent ‘overlap’ syndromes. All these forms of MEN may either be inherited as autosomal dominant syndromes or they may occur sporadically, i.e. without a family history. However, this distinction between sporadic and familial cases may sometimes be difficult as in some sporadic cases the family history may be absent because the parent with the disease may have died before developing symptoms. In this chapter, the main clinical features and molecular genetics of the MEN 1 syndrome will be discussed.

  • Multiple Endocrine Neoplasia type 1 (MEN1)
    European Journal of Cancer, 1994
    Co-Authors: J.t. Pang, R.v. Thakker
    Abstract:

    Multiple Endocrine Neoplasia (1–3) is characterized by the occurrence of tumors involving two or more Endocrine glands within a single patient. The disorder has previously been referred to as Multiple Endocrine adenopathy (MEA) or the pluriglandular syndrome. However, glandular hyperplasia and malignancy may also occur in some patients and the term Multiple Endocrine Neoplasia (MEN) is now preferred. There are two major forms of Multiple Endocrine Neoplasia referred to as type 1 and type 2 and each form is characterized by the development of tumors within specific Endocrine glands (see Table 1). Thus, the combined occurrence of tumors of the parathyroid glands, the pancreatic islet cells, and the anterior pituitary is characteristic of Multiple Endocrine Neoplasia type 1(MEN1), which is also referred to as Wermer’s syndrome. In addition to these tumors, adrenal cortical, carcinoid, facial angiofibromas, collagenomas, and lipomatous tumors have also been described in patients with MENI (3, 4). However, in Multiple Endocrine Neoplasia type 2 (MEN2), which is also called Sipple’s syndrome, medullary thyroid carcinoma (MTC) occurs in association with phaeochromocytoma, and three clinical variants referred to as MEN2a, MEN2b and MTC-only are recognized (1, 5). In MEN2a, which is the most common variant, the development of MTC is associated with phaeochromocytoma and parathyroid tumors. However, in MEN2b parathyroid involvement is absent and the occurrence of MTC and phaeochromocytoma is found in association with a marfanoid habitus mucosal neuromas, medullated corneal fibers and intestinal autonomic ganglion dysfunction leading to a megacolon.

  • Multiple Endocrine Neoplasia and Molecular Genetics
    Surgical Endocrinology, 1993
    Co-Authors: R.v. Thakker
    Abstract:

    Publisher Summary Multiple Endocrine Neoplasia is characterized by the occurrence of tumors involving two or more Endocrine glands within a single patient. The disorder has previously been referred to as Multiple Endocrine adenopathy or the pluriglandular syndrome. Glandular hyperplasia and malignancy may also occur in some patients and the term Multiple Endocrine Neoplasia is now preferred. There are two major forms of Multiple Endocrine Neoplasia referred to as type 1 and type 2 and each form is characterized by the development of tumors within specific Endocrine glands. Multiple Endocrine Neoplasia type 1 (MEN1), which is also referred to as Wermers syndrome, is characterized by the combined occurrence of tumors of the parathyroid glands, the pancreatic islet cells, and the anterior pituitary. The incidence of MEN1 has been estimated from randomly chosen post-mortem studies to be 0.25% and to be 18% among patients with primary hyperparathyroidism. The disorder affects all age groups, with a reported age range of 5–81 years, and 80% of patients have developed clinical manifestations of the disorder by the fifth decade. The clinical manifestations of MEN1 are related to the sites of tumors and to their secretion products.

Katherine E. Teague - One of the best experts on this subject based on the ideXlab platform.

  • Pregnancy complicated by Multiple Endocrine Neoplasia type IIA (Sipple's syndrome)
    American journal of obstetrics and gynecology, 1997
    Co-Authors: Joseph R. Wax, Maurice K. Eggleston, Katherine E. Teague
    Abstract:

    Abstract A patient with preexisting Multiple Endocrine Neoplasia type IIA had normal 24-hour urinary metanephrine and vanillylmandelic acid excretions before and during pregnancy. After a benign prenatal course, the patient had a term spontaneous vaginal delivery. Multiple Endocrine Neoplasia type IIA antedating pregnancy may be associated with a normal obstetric outcome in the absence of a pheochromocytoma. (Am J Obstet Gynecol 1997;177:461-2.)

Yoshitaka Fujii - One of the best experts on this subject based on the ideXlab platform.

  • ACTH-secreting thymic carcinoid associated with Multiple Endocrine Neoplasia type 1.
    The Annals of thoracic surgery, 2006
    Co-Authors: Motoki Yano, Ichiro Fukai, Yoshihiro Kobayashi, Kotaro Mizuno, Akimitsu Konishi, Hiroshi Haneda, Eriko Suzuki, Katsuhiko Endo, Yoshitaka Fujii
    Abstract:

    Thymic carcinoids are classified into three categories: (1) nonsecretory tumors, (2) hormonal secretory tumors, and (3) tumors associated with Multiple Endocrine Neoplasia type 1. We report a rare case with adrenocorticotropic hormone secreting thymic carcinoid with Multiple Endocrine Neoplasia type 1. Radiologic examination showed an anterior mediastinal mass and a parathyroid tumor. Blood analysis revealed high levels of parathyroid hormone and adrenocorticotropic hormone. Urine cortisol and 17-hydroxycorticoids levels were also elevated. Extended thymectomy was performed. Subsequently adjuvant radiation therapy and parathyroid tumor resection were performed. A germline mutation of exon 7 in the Multiple Endocrine Neoplasia type 1 gene was detected and a somatic mutation of exon 9 was demonstrated in the thymic tumor.

Michael A. Skinner - One of the best experts on this subject based on the ideXlab platform.

  • Multiple Endocrine Neoplasia Type 2
    Endocrine Surgery in Children, 2017
    Co-Authors: Eduardo A. Perez, Michael A. Skinner
    Abstract:

    Multiple Endocrine Neoplasia type 2 (MEN2) is a rare autosomal dominant disorder that predisposes patients to medullary thyroid cancer (MTC), pheochromocytoma (PHEO), and primary parathyroid hyperplasia (PHPT). MEN2 is subclassified into three distinct syndromes: Multiple Endocrine Neoplasia type 2A (MEN2A), Multiple Endocrine Neoplasia type 2B (MEN2B), and familial medullary thyroid cancer (FMTC). All three syndromes are the result of mutations of the RET proto-oncogene. The diagnosis and management of MEN2 syndromes in children is discussed.

  • Surgical intervention in children with Multiple Endocrine Neoplasia type 2.
    Current opinion in pediatrics, 2006
    Co-Authors: Melissa E. Danko, Michael A. Skinner
    Abstract:

    PURPOSE OF REVIEW We provide a summary of the literature published in the past year addressing the surgical approach to Multiple Endocrine Neoplasia type 2 in the pediatric population. RECENT FINDINGS The review focuses first on medullary thyroid carcinoma and performing prophylactic thyroidectomy for the prevention or cure of this disease. The timing and extent of surgery as well as additional surgical intervention for persistent or recurrent disease is discussed. Then the surgical management of hereditary pheochromocytoma is reviewed. SUMMARY Surgery is often the only treatment that can prevent or cure the endocrinopathies associated with Multiple Endocrine Neoplasia type 2. Determining the proper timing and extent of surgical intervention in children affected with Multiple Endocrine Neoplasia type 2 will lead to better outcomes and survival.

William H. Beierwaltes - One of the best experts on this subject based on the ideXlab platform.