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Multiple Endocrine Neoplasia Type 1

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101. Incidence and prevalence of multiple endocrine neoplasia 2B in Denmark: a nationwide study. (PubMed)

England Endocr Relat Cancer 9436481 1351-0088 IM Adolescent Adult Cohort Studies Denmark Female Humans Incidence Male Multiple Endocrine Neoplasia Type 2b epidemiology pathology Prevalence Retrospective Studies Young Adult 2017 04 19 2017 04 24 2017 4 26 6 0 2018 10 17 6 0 2017 4 26 6 0 ppublish 28438782 ERC170122 10.1530/ERC-17-0122 (...) Incidence and prevalence of multiple endocrine neoplasia 2B in Denmark: a nationwide study. 28438782 2018 10 16 2018 11 28 1479-6821 24 7 2017 07 Endocrine-related cancer Endocr. Relat. Cancer Incidence and prevalence of multiple endocrine neoplasia 2B in Denmark: a nationwide study. L39-L42 10.1530/ERC-17-0122 ERC170122 Mathiesen Jes Sloth JS Department of ORL Head & Neck SurgeryOdense University Hospital, Odense, Denmark. Institute of Clinical ResearchUniversity of Southern Denmark, Odense

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2017 Endocrine-Related Cancer

102. Genetics of Endocrine and Neuroendocrine Neoplasias (PDQ®): Health Professional Version

to the summary for more information about variant classification. ] There are several hereditary syndromes that involve endocrine or neuroendocrine glands, such as multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2 (MEN2), multiple endocrine neoplasia type 4 (MEN4), pheochromocytoma (PHEO), paraganglioma (PGL), Li-Fraumeni syndrome, familial adenomatous polyposis, and von Hippel-Lindau syndrome. This summary currently focuses on MEN1, MEN2, MEN4, familial PHEO and PGL syndrome (...) , et al.: Familial papillary carcinoma of the thyroid. Am J Med Genet 25 (4): 775-82, 1986. [ ] Loh KC: Familial nonmedullary thyroid carcinoma: a meta-review of case series. Thyroid 7 (1): 107-13, 1997. [ ] Lupoli G, Vitale G, Caraglia M, et al.: Familial papillary thyroid microcarcinoma: a new clinical entity. Lancet 353 (9153): 637-9, 1999. [ ] Multiple Endocrine Neoplasia Type 1 Clinical Description Multiple endocrine neoplasia type 1 (MEN1) ( ) is an autosomal dominant syndrome

2018 PDQ - NCI's Comprehensive Cancer Database

103. Multiple Endocrine Neoplasia Type 1 (Diagnosis)

Multiple Endocrine Neoplasia Type 1 (Diagnosis) Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1): Practice Essentials, Background, Pathophysiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA5MzcyMy1vdmVydmlldw== processing > Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Updated: Sep 14, 2018 Author: Thomas N Darling, MD, PhD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Overview Practice Essentials Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant familial tumor syndrome (also termed ) characterized by tumors of the parathyroid glands

2014 eMedicine.com

104. Multiple Endocrine Neoplasia Type 1 (Overview)

Multiple Endocrine Neoplasia Type 1 (Overview) Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1): Practice Essentials, Background, Pathophysiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA5MzcyMy1vdmVydmlldw== processing > Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Updated: Sep 14, 2018 Author: Thomas N Darling, MD, PhD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Overview Practice Essentials Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant familial tumor syndrome (also termed ) characterized by tumors of the parathyroid glands

2014 eMedicine.com

105. Multiple Endocrine Neoplasia Type 1 (Treatment)

Multiple Endocrine Neoplasia Type 1 (Treatment) Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management: Medical Care, Surgical Care, Consultations Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA5MzcyMy10cmVhdG1lbnQ= processing > Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment & Management Updated: Sep 14, 2018 Author: Thomas N Darling, MD, PhD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Treatment Medical Care Clinical practice guidelines have been developed based on a review of peer-review publications for managing MEN1 [ ] . Related clinical guideline

2014 eMedicine.com

106. Multiple Endocrine Neoplasia Type 1 (Follow-up)

Multiple Endocrine Neoplasia Type 1 (Follow-up) Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Follow-up: Complications, Patient Education Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMTA5MzcyMy1mb2xsb3d1cA== processing > Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Follow-up Updated: Sep 14, 2018 Author: Thomas N Darling, MD, PhD; Chief Editor: Dirk M Elston, MD Share Email Print Feedback Close Sections Sections Dermatologic Manifestations of Multiple Endocrine Neoplasia Type 1 (MEN1) Follow-up Complications Endocrine tumors in MEN1 Tumors may hypersecrete hormone, causing hypercalcemia and recurrent nephrolithiasis (hyperparathyroidism), Zollinger-Ellison syndrome

2014 eMedicine.com

108. Primary hyperparathyroidism in young people. When should we perform genetic testing for multiple endocrine neoplasia 1 (MEN-1)? (PubMed)

was incidentally found also to have hypercalcemia and elevated PTH (pHPT). Exploratory neck surgery showed multiglandular parathyroid affection; she turned out to have MEN-1, but she was diagnosed 7 years after her debut of pHPT.The aim was to search literature on indications for performing mutational analysis in young patients with pHPT and no family history of MEN-1. PubMed was searched for English language articles, and words used were: MEN1 OR MEN-1 OR MEN type 1 OR multiple endocrine neoplasia 1 (...) OR multiple endocrine neoplasia type 1 AND Mutational analysis OR genetic testing OR testing OR Hyperparathyroidism, primary [majr]. A total of 625 abstracts were reviewed.Whether to perform screening of patients with pHPT under the age of 30, 35, or 40 years is controversial. According to international guidelines from 2001, genetic testing is indicated only in patients with pHPT below the age of 30 years. However, in updated guidelines from 2012, it is suggested to perform genetic testing in patients

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2014 Journal of Clinical Endocrinology and Metabolism

109. Local recurrence of pheochromocytoma in multiple endocrine neoplasia type 2A: a diagnostic and therapeutic challenge (PubMed)

Local recurrence of pheochromocytoma in multiple endocrine neoplasia type 2A: a diagnostic and therapeutic challenge In a patient with multiple endocrine neoplasia type 2A (MEN2A), an inverted physiological ratio between urinary normetanephrines and metanephrines is an early marker of recurrence in epinephrine-secreting pheochromocytoma, and 131I MIBG treatment appears to be a useful therapeutic option in order to avoid multiple invasive surgical procedures in pheochromocytomatosis.

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2016 Clinical Case Reports

110. Genetic analysis and clinical investigation of a pedigree with multiple endocrine neoplasia type 2A: A case report (PubMed)

Genetic analysis and clinical investigation of a pedigree with multiple endocrine neoplasia type 2A: A case report Multiple endocrine neoplasia 2A (MEN2A) is characterized by the coexistence of tumors that involve two or more endocrine glands within the same patient, and is defined as the occurrence of medullary thyroid carcinoma in association with pheochromocytoma (PHEO) and parathyroid tumors or hyperparathyroidism. The pathogenesis of MEN2A is due to the mutation of a tyrosine kinase

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2016 Oncology letters

111. Multiple endocrine neoplasia phenocopy revealed as a co‐occurring neuroendocrine tumor and familial hypocalciuric hypercalcemia type 3 (PubMed)

Multiple endocrine neoplasia phenocopy revealed as a co‐occurring neuroendocrine tumor and familial hypocalciuric hypercalcemia type 3 Familial hypocalciuric hypercalcemia type 3 should be considered as differential diagnosis in patients with suspected primary hyperparathyroidism and/or suspected multiple neoplasia syndrome, as correct diagnosis will spare the patients for going through multiple futile parathyroidectomies and for the worry of being diagnosed with a cancer susceptibility

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2016 Clinical Case Reports

112. Recurrent hyperparathyroidism due to proliferation of autotransplanted parathyroid tissue in a multiple endocrine neoplasia type 2A patient (PubMed)

Recurrent hyperparathyroidism due to proliferation of autotransplanted parathyroid tissue in a multiple endocrine neoplasia type 2A patient About 20%-30% of all cases of multiple endocrine neoplasia type 2A (MEN 2A) is accompanied by primary hyperparathyroidism. These patients undergo parathyroidectomy and, if needed, autotransplantation. In rare cases, autotransplanted parathyroid tissues can cause hypoparathyroidism due to failure of transplantation or hyperparathyroidism due to proliferation

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2016 Annals of surgical treatment and research

113. Positive Germline Selection in Pedigrees With Multiple Endocrine Neoplasia Type 2 Carrying V804M Mutation in the RET Gene (PubMed)

Positive Germline Selection in Pedigrees With Multiple Endocrine Neoplasia Type 2 Carrying V804M Mutation in the RET Gene Multiple endocrine neoplasia (MEN) type 2 is an autosomal dominant cancer syndrome associated with the development of thyroid cancer and tumors or hyperplasia in other endocrine organs. It is caused by mutations in the RET gene and can be phenotypically classified into MEN types 2A and 2B. MEN2B is often sporadic resulting from a spontaneous mutation, M981T. A positive

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2016 World journal of oncology

114. Perioperative Severe Hypotension in a Patient with Multiple Endocrine Neoplasia Type IIb and Bilateral Adrenalectomies: Time to Review the Evidence for Stress Dose Steroids (PubMed)

Perioperative Severe Hypotension in a Patient with Multiple Endocrine Neoplasia Type IIb and Bilateral Adrenalectomies: Time to Review the Evidence for Stress Dose Steroids Multiple endocrine neoplasia type IIb (MEN IIb) is an endocrine disorder which can manifest with tumors such as pheochromocytomas and neuromas. We present the case of a patient with MEN IIb, after bilateral adrenalectomies, on maintenance steroid replacement, who underwent a neuroma resection and developed severe hypotension

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2016 Case reports in anesthesiology

115. A Nationwide study of multiple endocrine neoplasia type 2A in Norway. Predictive avd prognostic factors for the clinical course of medullary thyroid carcinoma. (PubMed)

A Nationwide study of multiple endocrine neoplasia type 2A in Norway. Predictive avd prognostic factors for the clinical course of medullary thyroid carcinoma. Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant syndrome caused by activating germline mutations in the RET (REarranged during Transfection) proto-oncogene. MEN 2A has a strong (>95%) and age-dependent (5-25 years) clinical penetrance of medullary thyroid carcinoma (MTC). Several major studies have analyzed (...) in Norway since 1974. Data were collected by reviewing patient files. The variables analyzed were genotype, phenotype, preoperative basal calcitonin, age at thyroid surgery, central lymph node dissection and nodal status at primary surgery, number of surgical procedures, and biochemical cure. Of the 65 patients, 60 had undergone thyroid surgery. The median follow-up period was 9.9 years. The patients were divided into pre-RET-and RET-era, which included patients who had thyroid surgery before January 1

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2016 Thyroid

116. Genetics of Endocrine and Neuroendocrine Neoplasias (PDQ®): Health Professional Version

to the summary for more information about variant classification. ] There are several hereditary syndromes that involve endocrine or neuroendocrine glands, such as multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2 (MEN2), multiple endocrine neoplasia type 4 (MEN4), pheochromocytoma (PHEO), paraganglioma (PGL), Li-Fraumeni syndrome, familial adenomatous polyposis, and von Hippel-Lindau syndrome. This summary currently focuses on MEN1, MEN2, MEN4, familial PHEO and PGL syndrome (...) , et al.: Familial papillary carcinoma of the thyroid. Am J Med Genet 25 (4): 775-82, 1986. [ ] Loh KC: Familial nonmedullary thyroid carcinoma: a meta-review of case series. Thyroid 7 (1): 107-13, 1997. [ ] Lupoli G, Vitale G, Caraglia M, et al.: Familial papillary thyroid microcarcinoma: a new clinical entity. Lancet 353 (9153): 637-9, 1999. [ ] Multiple Endocrine Neoplasia Type 1 Clinical Description Multiple endocrine neoplasia type 1 (MEN1) ( ) is an autosomal dominant syndrome

2016 PDQ - NCI's Comprehensive Cancer Database

117. A rare presentation of multiple endocrine neoplasia (MEN) type 2A syndrome (PubMed)

A rare presentation of multiple endocrine neoplasia (MEN) type 2A syndrome Peptic ulcer disease may be a manifestation of symptomatic primary hyperparathyroidism. A case of an intractable complicated peptic ulcer disease secondary to hypercalcaemia from multiple endocrine neoplasia type 2A is presented. Hypercalcaemia should always be excluded as a cause of recurrent, or complicated peptic ulcer disease.

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2015 Annals of Medicine and Surgery

118. Management of the Parathyroid Glands During Preventive Thyroidectomy in Patients With Multiple Endocrine Neoplasia Type 2. (PubMed)

Management of the Parathyroid Glands During Preventive Thyroidectomy in Patients With Multiple Endocrine Neoplasia Type 2. Patients with multiple endocrine neoplasia type 2 (MEN2) have mutations in the RET protooncogene and virtually all of them will develop medullary thyroid carcinoma. Family members identified by genetic testing are candidates for preventive thyroidectomy. Management of the parathyroids during thyroidectomy is controversial. Some experts advocate total parathyroidectomy (...) (group B). Individual parathyroids were autotransplanted only if they appeared nonviable or could not be preserved intact. Central neck dissection was done only if the serum calcitonin was greater than 40 pg/mL.Permanent hypoparathyroidism occurred in 3 (6%) of 50 patients in group A, compared with 1 (1%) of 102 patients in group B (P = 0.1). After total thyroidectomy, no patient in either group developed permanent recurrent laryngeal nerve injury or hyperparathyroidism. Immediate postoperative serum

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2015 Annals of Surgery

119. Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study. (PubMed)

Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study. The prevention of medullary thyroid cancer in patients with multiple endocrine neoplasia type 2 syndrome has demonstrated the ability of molecular diagnosis and prophylactic surgery to improve patient outcomes. However, the other major neoplasia associated with multiple endocrine neoplasia type 2 (...) , phaeochromocytoma, is not as well characterised in terms of occurrence and treatment outcomes. In this study, we aimed to systematically characterise the outcomes of management of phaeochromocytoma associated with multiple endocrine neoplasia type 2.This multinational observational retrospective population-based study compiled data on patients with multiple endocrine neoplasia type 2 from 30 academic medical centres across Europe, the Americas, and Asia. Patients were included if they were carriers of germline

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2014 Lancet Oncology

120. RET Cys634Arg mutation confers a more aggressive multiple endocrine neoplasia type 2A phenotype than Cys634Tyr mutation. (PubMed)

RET Cys634Arg mutation confers a more aggressive multiple endocrine neoplasia type 2A phenotype than Cys634Tyr mutation. Specific germline mutations in the RET proto-oncogene are correlated with clinical features in multiple endocrine neoplasia type 2A (MEN2A); however, data are scarce regarding differences in clinical profiles dependent on the type of nucleotide and amino acid substitution at the same codon. We aimed to analyse differences in clinical risk profiles and outcomes among different (...) amino acids encoded by codon 634.The study was retrospective and multicentric.We collected data included in the Spanish Online National Database from patients with MEN2A carrying a RET proto-oncogene mutation on codon 634. The mean follow-up time was 7.6±6.9 years (1-32).Patients (n=173) from 49 unrelated families were C634Y carriers, and 26 patients from eight different families had C634R mutation. We found higher penetrance of medullary thyroid carcinoma, phaeochromocytoma and hyperparathyroidism

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2014 European Journal of Endocrinology

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