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Multiple Endocrine Neoplasia Type 1

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61. Incidence and prevalence of multiple endocrine neoplasia 2A in Denmark 1901–2014: a nationwide study (PubMed)

Incidence and prevalence of multiple endocrine neoplasia 2A in Denmark 1901–2014: a nationwide study The incidence and prevalence of multiple endocrine neoplasia 2A (MEN2A) have only been reported once in a nationwide setting. However, it is unclear whether the figures are representative of other populations, as the major component of the syndrome, hereditary medullary thyroid carcinoma (MTC), has been reported as rare in the same country. We conducted a nationwide retrospective cohort study (...) million live births per year. Incidence for the specific mutations or for the overall MEN2A group did not change significantly from 1901 to 2014 (P>0.05). Point prevalence at January 1, 2015, was 24 per million (95% CI: 20-28).The incidence and prevalence of MEN2A in Denmark seem higher than those reported in other countries. This is likely explained by the Danish C611Y founder effect. Also, our data indicate no significant change in MEN2A incidence during the last century.

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2018 Clinical epidemiology

62. Multiple Endocrine Neoplasia Syndromes from Genetic and Epigenetic Perspectives (PubMed)

Multiple Endocrine Neoplasia Syndromes from Genetic and Epigenetic Perspectives Multiple endocrine neoplasia (MEN) syndromes are infrequent inherited disorders in which more than one endocrine glands develop noncancerous (benign) or cancerous (malignant) tumors or grow excessively without forming tumors. There are 3 famous and well-known forms of MEN syndromes (MEN 1, MEN 2A, and MEN 2B) and a newly documented one (MEN4). These syndromes are infrequent and occurred in all ages and both men (...) and women. Usually, germ line mutations that can be resulted in neoplastic transformation of anterior pituitary, parathyroid glands, and pancreatic islets in addition to gastrointestinal tract can be an indicator for MEN1. The medullary thyroid cancer (MTC) in association with pheochromocytoma and/or multiple lesions of parathyroid glands with hyperparathyroidism can be pointer of MEN2 which can be subgrouped into the MEN 2A, MEN 2B, and familial MTC syndromes. There are no distinct biochemical markers

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2018 Biomarker insights

63. Multiple endocrine neoplasia 2 in Cyprus: evidence for a founder effect (PubMed)

Multiple endocrine neoplasia 2 in Cyprus: evidence for a founder effect Multiple endocrine neoplasia type 2 (MEN2) affects patients with RET proto-oncogene mutations. This cohort study refers to patients who were diagnosed with familial medullary thyroid carcinoma (MTC) and underwent RET genetic testing in Cyprus between years 2002 and 2017.Forty patients underwent RET testing by Sanger sequencing of exons 10-11 and 13-16. Genotyping with STR genetic markers flanking the RET gene along with Y

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2018 Journal of endocrinological investigation

64. Metastasis of carcinoid to the arch of the axis in a multiple endocrine neoplasia patient: a case report. (PubMed)

Metastasis of carcinoid to the arch of the axis in a multiple endocrine neoplasia patient: a case report. Carcinoid tumors eventually metastasize to the spine, and epidural spinal cord compression is a relatively frequent neurologic complication of carcinoid. However, a case of multiple endocrine neoplasia type 1 (MEN1) presenting with spinal cord compression as a result of a metastatic carcinoid tumor has not been reported previously.To report an extremely rare case of MEN1 presenting (...) metastasis of carcinoid tumor occurred in a multiple endocrine neoplasia patient, and it is significant to note that carcinoid metastasis is one of differential diagnoses for osteoblastic lesions.

2017 The Spine Journal

65. Multiple Endocrine Neoplasia Type 2

Multiple Endocrine Neoplasia Type 2 Multiple Endocrine Neoplasia Type 2 Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Multiple (...) Endocrine Neoplasia Type 2 Multiple Endocrine Neoplasia Type 2 Aka: Multiple Endocrine Neoplasia Type 2 , Multiple Endocrine Neoplasia Type II , Sipple's Syndrome , MEN II From Related Chapters II. Associated Conditions Multiple Endocrine Neoplasia Type 2 or Adrenal adenoma carcinoma Multiple Endocrine Neoplasia Type 2-A ry tumor III. Prevention: Screening in family members Identify specific gene mutation in known positive family member with MEN II Screen family members for the specific gene mutation IV

2018 FP Notebook

66. No Association of Blood Type O with Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1. (PubMed)

No Association of Blood Type O with Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1. An association between ABO blood type and the development of cancer, in particular, pancreatic cancer, has been reported in the literature. An association between blood type O and neuroendocrine tumors in multiple endocrine neoplasia type 1 (MEN1) patients was recently suggested. Therefore, blood type O was proposed as an additional factor to personalize screening criteria for neuroendocrine tumors (...) in MEN1 patients.The aim of this study was to assess the association between blood type O and the occurrence of neuroendocrine tumors in the national Dutch MEN1 cohort.This is a cohort study using the Dutch National MEN1 database, which includes more than 90% of the Dutch MEN1 population. Demographic and clinical data were analyzed by blood type. Chi-square tests and Fisher exact tests were used to determine the association between blood type O and occurrence of neuroendocrine tumors. A cumulative

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2015 Journal of Clinical Endocrinology and Metabolism

67. Imaging in multiple endocrine neoplasia type 1: recent studies show enhanced sensitivities but increased controversies (PubMed)

Imaging in multiple endocrine neoplasia type 1: recent studies show enhanced sensitivities but increased controversies In multiple endocrine neoplasia type 1 (MEN1) patients, a number of recent studies compare the ability of different, new imaging modalities to existing modalities to localize the important neuroendocrine tumors (NETs) that contribute to their decreased life expectancy (pancreatic NETs [pNETs] and thymic carcinoids). These included the use of 68Ga-DOTATOC-PET/CT, endoscopic

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2016 International journal of endocrine oncology

68. Multiple Endocrine Neoplasia Type 1 Presenting as Hypoglycemia due to Insulinoma (PubMed)

Multiple Endocrine Neoplasia Type 1 Presenting as Hypoglycemia due to Insulinoma Multiple endocrine neoplasia (MEN) mutation is an autosomal dominant disorder characterized by the occurrence of parathyroid, pancreatic islet, and anterior pituitary tumors. The incidence of insulinoma in MEN is relatively uncommon, and there have been a few cases of MEN manifested with insulinoma as the first symptom in children. We experienced a 9-year-old girl having a familial MEN1 mutation. She complained

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2016 Journal of Korean medical science

69. Multiple endocrine neoplasia type 1 with anterior mediastinal parathyroid adenoma: successful localization using Tc-99m sestamibi SPECT/CT (PubMed)

Multiple endocrine neoplasia type 1 with anterior mediastinal parathyroid adenoma: successful localization using Tc-99m sestamibi SPECT/CT The most common manifestation of multiple endocrine neoplasia type 1 (MEN1) is hyperparathyroidism. Treatment of hyperparathyroidism in MEN patients is surgical removal of the parathyroid glands, however ectopic parathyroid gland is challenging for treatment. A 51-year-old female, the eldest of 3 MEN1 sisters, had hyperparathyroidism with ectopic parathyroid

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2016 Annals of surgical treatment and research

70. Concomitant existence of pheochromocytoma in a patient with multiple endocrine neoplasia type 1 (PubMed)

Concomitant existence of pheochromocytoma in a patient with multiple endocrine neoplasia type 1 Multiple endocrine neoplasia type 1 (MEN1) is an autosomal-dominant inherited disorder that is classically characterized by the presence of neoplastic lesions of the parathyroid glands, the anterior pituitary gland, and the pancreas. However, MEN1 with concomitant pheochromocytoma is extremely rare.We report a case of MEN1 concomitant with pheochromocytoma. A 44-year-old Japanese man, who had (...) undergone total parathyroidectomy due to primary hyperparathyroidism at the age of 18, was referred to our hospital with a complaint of a large abdominal tumor. He was diagnosed as having a giant insulinoma (maximum diameter 18 cm) in the pancreatic tail, five other non-functional neuroendocrine tumors in the pancreatic body and tail, multiple liver metastases of pancreatic neuroendocrine tumors, a pituitary prolactinoma, non-functional adrenal cortical adenomas, a pheochromocytoma in addition

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2016 Surgical Case Reports

71. Lung adenocarcinoma and adrenocortical carcinoma in a patient with multiple endocrine neoplasia type 1 (PubMed)

Lung adenocarcinoma and adrenocortical carcinoma in a patient with multiple endocrine neoplasia type 1 Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder caused by heterozygous germline mutations in the tumor suppressor gene MEN1, which encodes a nuclear protein, menin. MEN1 is characterized by the combined occurrence of tumors involving the pituitary gland, pancreatic islets, and parathyroid glands. Additionally, patients with MEN1 often exhibit adrenal tumors (...) . Although most MEN1-associated tumors are benign, malignant lesions arising in these endocrine organs have been reported. Additionally, malignant diseases of non-endocrine organs concomitant with MEN1 have also been reported. Here, we report a rare case of a MEN1 patient who exhibited adrenocortical carcinoma (ACC) and lung adenocarcinoma (LAC). A 53-year-old Japanese woman was diagnosed with genetically proven MEN1 that initially manifested as parathyroid, pancreatic, and adrenal tumors. During

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2016 Respiratory Medicine Case Reports

72. Utility of chromogranin A, pancreatic polypeptide, glucagon, and gastrin in the diagnosis and follow-up of pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 patients (PubMed)

Utility of chromogranin A, pancreatic polypeptide, glucagon, and gastrin in the diagnosis and follow-up of pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 patients Pancreatic neuroendocrine tumours (PNETs) are the major source of disease-specific mortality in multiple endocrine neoplasia type 1 (MEN1) patients. Chromogranin A (CgA), pancreatic polypeptide (PP), glucagon and gastrin have some diagnostic value in sporadic PNETs, but there is very little evidence (...) MEN1 cases, 55 PNETs and 58 non-PNETs met inclusion criteria. The area under the curve (AUC) for CgA, PP, glucagon and gastrin in MEN1 cases was 59·5%, 64·1%, 77·0% and 75·9%, respectively. The AUC for the combination of CgA, PP and gastrin was 59·6%. PP, but not CgA, glucagon or gastrin was significantly associated with both age and PNET functional status (P = 0·0485 and 0·0188, respectively). No markers were significantly associated with sex, PNET size, tumour number, tumour location, American

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2016 Clinical endocrinology

73. Assessing for multiple endocrine neoplasia type 1 in patients evaluated for Zollinger-Ellison Syndrome - clues to a safer diagnostic process. (PubMed)

Assessing for multiple endocrine neoplasia type 1 in patients evaluated for Zollinger-Ellison Syndrome - clues to a safer diagnostic process. Zollinger-Ellison syndrome is a rare cause of tumoral hypergastrinemia; 1 of 5 patients with this syndrome also has multiple endocrine neoplasia type 1. The diagnosis of this disease is complicated by the widespread use of proton pump inhibitors that can elevate serum gastrin levels, the cornerstone for biochemical diagnosis. Abrupt discontinuation (...) of proton pump inhibitors could lead to adverse outcomes. Clinician awareness of the relationship between Zollinger-Ellison syndrome and multiple endocrine neoplasia type 1 could lead to a safer diagnostic pathway.We conducted a retrospective review of a cohort of patients with multiple endocrine neoplasia type 1.There were 287 patients with multiple endocrine neoplasia type 1 (73 with gastrinoma) evaluated between 1997 and 2014. Two patients experienced adverse events after proton pump inhibitor

2016 American Journal of Medicine

74. Growth rate of small pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: results from an endoscopic ultrasound based cohort study. (PubMed)

Growth rate of small pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: results from an endoscopic ultrasound based cohort study. Background and aims In multiple endocrine neoplasia type 1 (MEN1), endoscopic ultrasound (EUS) is used for identification and follow-up of pancreatic neuroendocrine tumors (PNETs). The role of EUS in surveillance of small ( < 20 mm) PNETs is unclear, mostly because the natural course of these lesions is largely unknown. We aimed to determine

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2016 Endoscopy

75. Reoperative Surgery in Patients with Multiple Endocrine Neoplasia Type 1 Associated Primary Hyperparathyroidism. (PubMed)

Reoperative Surgery in Patients with Multiple Endocrine Neoplasia Type 1 Associated Primary Hyperparathyroidism. Persistent/recurrent primary hyperparathyroidism (pHPT) occurs frequently in multiple endocrine neoplasia type 1 (MEN1). We assessed the usefulness of intraoperative PTH (IOPTH) and preoperative localizing studies based on the outcome of patients with MEN1-associated pHPT undergoing reoperative surgery.A retrospective analysis identified MEN1 patients with persistent/recurrent pHPT

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2016 Annals of Surgical Oncology

76. Unilateral Clearance for Primary Hyperparathyroidism in Selected Patients with Multiple Endocrine Neoplasia Type 1. (PubMed)

Unilateral Clearance for Primary Hyperparathyroidism in Selected Patients with Multiple Endocrine Neoplasia Type 1. Primary hyperparathyroidism is the most common manifestation of multiple endocrine neoplasia type 1 (MEN1). Guidelines advocate subtotal parathyroidectomy (STP) or total parathyroidectomy with autotransplantation due to high prevalence of multiglandular disease; however, both are associated with a significant risk of permanent hypoparathyroidism. More accurate imaging and use

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2016 World Journal of Surgery

77. Pasireotide therapy of Multiple Endocrine Neoplasia type 1 (MEN1)-associated neuroendocrine tumors (NETs) in female mice deleted for an Men1 allele (Men1<sup>+/-</sup>) improves survival and reduces tumor progression. (PubMed)

Pasireotide therapy of Multiple Endocrine Neoplasia type 1 (MEN1)-associated neuroendocrine tumors (NETs) in female mice deleted for an Men1 allele (Men1+/-) improves survival and reduces tumor progression. Pasireotide, a somatostatin analog, is reported to have anti-proliferative effects in neuroendocrine tumors (NETs). We therefore assessed the efficacy of pasireotide for treating pancreatic and pituitary NETs that develop in a mouse model of multiple endocrine neoplasia type 1 (...) (MEN1). Men1(+/-) mice were treated from age 12 mo with 40 mg/kg pasireotide long-acting release formulation, or PBS, intramuscularly monthly for 9 mo. The Men1(+/-) mice had magnetic resonance imaging at 12 and 21 mo, and from 20 mo oral 5-bromo-2-deoxyuridine for 1 mo, to assess tumor development and proliferation, respectively. NETs were collected at age 21 mo, and proliferation and apoptosis assessed by immunohistochemistry and TUNEL assays, respectively. Pasireotide-treated Men1(+/-) mice had

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2016 Endocrinology

78. Type 1 Multiple Endocrine Neoplasia Cohort Study

Type 1 Multiple Endocrine Neoplasia Cohort Study Type 1 Multiple Endocrine Neoplasia Cohort Study - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Type 1 Multiple Endocrine Neoplasia Cohort Study (MEN1 (...) Information provided by (Responsible Party): Centre Hospitalier Universitaire Dijon Study Details Study Description Go to Brief Summary: Type 1 - Multiple Endocrine Neoplasia syndrome (MEN1,) is an autosomal dominant disorder secondary to MEN1 mutations that predisposes carriers to endocrine tumors. The MEN1 gene located on chromosome 11q13 encodes menin, a 610 amino acid protein expressed in all tissues tested. Menin is a scaffold protein which interacts with a large number of intracellular molecules

2016 Clinical Trials

79. Minimally Invasive Versus Open Pancreatic Surgery in Patients with Multiple Endocrine Neoplasia Type 1. (PubMed)

Minimally Invasive Versus Open Pancreatic Surgery in Patients with Multiple Endocrine Neoplasia Type 1. The role of minimally invasive pancreatic surgery for pancreatic neuroendocrine neoplasms (pNENs) in patients with multiple endocrine neoplasia type 1 (MEN1) is not well defined. The aim of this study was to compare the outcome of minimally invasive versus open pancreatic resections in patients with MEN1.Prospectively collected data of MEN1 patients who underwent a primary distal pancreatic (...) resection and/or enucleation for non-functioning pNENs or insulinoma were retrospectively analyzed regarding the outcome of minimally invasive or open pancreatic resections.Thirty-three patients underwent primary pancreatic resection for either organic hyperinsulinism (n = 9, 27 %) or non-functioning pNENs >1 cm in size (n = 24, 73 %) between 1987 and 2015. 21 (64 %) patients underwent an open surgical (group 1) and 12 patients (36 %) a minimally invasive approach, either laparoscopic (n = 8) or robotic

2016 World Journal of Surgery

80. A Possible New Multiple Endocrine Neoplasia Mutation in a Patient with a Prototypic Multiple Endocrine Neoplasia Presentation (PubMed)

A Possible New Multiple Endocrine Neoplasia Mutation in a Patient with a Prototypic Multiple Endocrine Neoplasia Presentation Multiple endocrine neoplasia (MEN) type 1 syndrome is an uncommon inherited disorder characterized by the occurrence of tumors involving two or more endocrine glands. These tumors include pheochromocytoma, adrenal cortical and neuroendocrine tumors including (bronchopulmonary, thymic, gastric), lipomas, angiofibromas, collagenomas, and meningiomas. MEN-4 is very rare (...) and has been characterized by the occurrence of parathyroid and anterior pituitary tumors in association with tumors of the adrenals, kidneys, and reproductive organs.We report the case of a 40-year-old male without significant family history of endocrine disease who was found to have primary hyperparathyroidism, a pituitary tumor causing acromegaly, thyroid cancer, renal cell carcinoma, and pancreatic cysts. We posit that this represents a new version of MEN-4. While renal tumors (angiomyolipoma

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2016 Cardiorenal medicine

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