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Multiple Endocrine Neoplasia Type 1

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141. Tanycytic ependymoma of the filum terminale associated with multiple endocrine neoplasia Type 1: first reported case. (PubMed)

Tanycytic ependymoma of the filum terminale associated with multiple endocrine neoplasia Type 1: first reported case. Ependymoma associated with multiple endocrine neoplasia type 1 (MEN-1) is an extremely rare clinical entity. To the best of our knowledge, only five cases of ependymoma associated with MEN-1 have been previously described. Furthermore, there has been no case of tanycytic ependymoma of the filum terminale associated with MEN-1.The present case report illustrates a 53-year-old man (...) with tanycytic ependymoma of the filum terminale associated with MEN-1. We review the literature on ependymoma with MEN-1 and tanycytic ependymoma of the cauda equina region and also discuss the risk of recurrence.A case report.The patient presented with complaints of nocturnal pain in the lower back, accompanied by numbness around the anus and intermittent claudication for approximately 1 year. Magnetic resonance imaging (MRI) identified an intradural-enhancing, large mass lesion at the level from Th12

2013 The Spine Journal

142. Case 200: gastric enterochromaffinlike cell tumors in a patient with type 1 multiple endocrine neoplasia. (PubMed)

Case 200: gastric enterochromaffinlike cell tumors in a patient with type 1 multiple endocrine neoplasia. History A 55-year-old man presented with chronic epigastric pain lasting for about 1 year and without fever or vomiting. The abdomen was soft and tender at physical examination. Laboratory tests revealed unremarkable liver function, normal hemoglobin level, and normal amylase level. White blood cell count was normal, and there was no inflammatory syndrome. The patient's medical history (...) included pancreatic gastrinoma resected by means of left pancreatectomy 31 years before, hyperparathyroidism treated with subtotal parathyroidectomy 24 years before, and a slowly growing lung mass known for 9 years. Esophagogastroduodenoscopy was performed because of a suspected gastroduodenal ulcer. The results showed numerous small (<10 mm) gastric and duodenal ulcers and multiple 10-15-mm polypoid gastric masses. Contrast material-enhanced dual-phase multidetector row computed tomography (CT

2013 Radiology

143. Multiple endocrine neoplasia type 1- presenting multiple lipomas and hypoglycemia onset (PubMed)

Multiple endocrine neoplasia type 1- presenting multiple lipomas and hypoglycemia onset Multiple endocrine neoplasia type 1 (MEN1), also called Wermer syndrome, is an autosomal dominant disorder characterized by tumors of the parathyroid glands, the anterior pituitary, and the endocrine pancreas.Here, we report a case of MEN1. Our patient was a 44-year-old woman who manifested typical features of MEN1, including insulinoma, pituitary tumors, and parathyroidoma, and exhibited multiple lipomas (...) and negative for glucagon.This case suggests that multiple lipomas, insulinoma and gastrinoma may provide clues for a diagnosis of MEN1.

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2012 The American journal of case reports

144. Association of Type-O Blood with Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1. (PubMed)

Association of Type-O Blood with Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1. The ABO blood type system describes the expression of human blood group antigens found on both erythrocytes and normal tissue throughout the body. We recently reported an association between O blood type and the manifestation of pancreatic neuroendocrine tumors in a cohort of patients with Von Hippel-Lindau syndrome.The aim of the study was to determine whether there is an association of ABO blood (...) type with the development of neuroendocrine tumors in patients with multiple endocrine neoplasia, type 1 (MEN-1).A retrospective analysis of 105 patients with MEN-1 was performed. Demographic, clinical, and biochemical data were analyzed by ABO blood type. Fisher's exact test was used to determine association between ABO blood type and manifestation of neuroendocrine tumor.Demographic and clinical characteristics were similar amongst blood type cohorts. We found an association between O blood type

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2012 Journal of Clinical Endocrinology and Metabolism

145. Post-surgical follow-up of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1 (PubMed)

Post-surgical follow-up of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1 The bone mineral density increments in patients with sporadic primary hyperparathyroidism after parathyroidectomy have been studied by several investigators, but few have investigated this topic in primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Further, as far as we know, only two studies have consistently evaluated bone mineral density values after (...) parathyroidectomy in cases of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Here we revised the impact of parathyroidectomy (particularly total parathyroidectomy followed by autologous parathyroid implant into the forearm) on bone mineral density values in patients with primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Significant increases in bone mineral density in the lumbar spine and femoral neck values were found, although no short-term (15

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2012 Clinics

146. Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? (PubMed)

Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? Diagnosis of multiple endocrine neoplasia type 1 (MEN1) is commonly based on clinical criteria, and confirmed by genetic testing. In patients without known MEN1-related germline mutations, the possibility of a casual association between two or more endocrine tumors cannot be excluded and subsequent management may be difficult to plan. We describe a very uncommon (...) pHPT were diagnosed and, since family history was negative, sporadic MEN1 was suspected. However, genetic testing revealed neither MEN-1 nor other gene mutations responsible for rarer cases of MEN1 (CDKN1B/p27 and other cyclin-dependent kinase inhibitor genes CDKN1A/p15, CDKN2C/p18, CDKN2B/p21). The patient underwent distal splenopancreatectomy and at the 4-month follow-up she showed complete remission of symptoms. Six months later, a thyroid nodule, suspected to be a malignant neoplasia, and two

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2012 BMC Cancer

147. Preoperative Assessment of the Pancreas in Multiple Endocrine Neoplasia Type 1. (PubMed)

Preoperative Assessment of the Pancreas in Multiple Endocrine Neoplasia Type 1. Many serologic and radiographic modalities are used for monitoring multiple endocrine neoplasia type 1 (MEN 1) patients for pancreaticoduodenal neuroendocrine tumors (PNETs). We compared serum markers and imaging studies obtained preoperatively with the gross pathology and immunohistochemical findings and correlated preoperative testing with postoperative outcome.From 2000 to 2008, 52 MEN 1 patients [32 (62%) female (...) ). Overall survival was 89% at 5-year follow-up.Our study substantiates EUS as providing the highest preoperative sensitivity and PPV in assessing the presence of PNETs in MEN 1 patients. CT and octreotide scintigraphy can yield both false-positive and false-negative results. HPP, gastrin, and glucagon were the most commonly measured tumor markers in our series but did not always correlate with immunostaining. With an aggressive surgical approach, satisfactory rates of biochemical improvement and long

2012 World Journal of Surgery

148. Clinical Practice Guidelines for Multiple Endocrine Neoplasia Type 1 (MEN1). (PubMed)

Clinical Practice Guidelines for Multiple Endocrine Neoplasia Type 1 (MEN1). The aim was to provide guidelines for evaluation, treatment, and genetic testing for multiple endocrine neoplasia type 1 (MEN1).The group, which comprised 10 experts, including physicians, surgeons, and geneticists from international centers, received no corporate funding or remuneration.Guidelines were developed by reviews of peer-reviewed publications; a draft was prepared, reviewed, and rigorously revised at several (...) pituitary tumors. Some patients may also develop carcinoid tumors, adrenocortical tumors, meningiomas, facial angiofibromas, collagenomas, and lipomas. Patients with MEN1 have a decreased life expectancy, and the outcomes of current treatments, which are generally similar to those for the respective tumors occurring in non-MEN1 patients, are not as successful because of multiple tumors, which may be larger, more aggressive, and resistant to treatment, and the concurrence of metastases. The prognosis

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2012 Journal of Clinical Endocrinology and Metabolism

149. Biochemical, bone and renal patterns in hyperparathyroidism associated with multiple endocrine neoplasia type 1. (PubMed)

Biochemical, bone and renal patterns in hyperparathyroidism associated with multiple endocrine neoplasia type 1. Primary hyperparathyroidism associated with multiple endocrine neoplasia type I (hyperparathyroidism/multiple endocrine neoplasia type 1) differs in many aspects from sporadic hyperparathyroidism, which is the most frequently occurring form of hyperparathyroidism. Bone mineral density has frequently been studied in sporadic hyperparathyroidism but it has very rarely been examined (...) in cases of hyperparathyroidism/multiple endocrine neoplasia type 1. Cortical bone mineral density in hyperparathyroidism/multiple endocrine neoplasia type 1 cases has only recently been examined, and early, severe and frequent bone mineral losses have been documented at this site. Early bone mineral losses are highly prevalent in the trabecular bone of patients with hyperparathyroidism/multiple endocrine neoplasia type 1. In summary, bone mineral disease in multiple endocrine neoplasia type 1 related

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2012 Clinics (São Paulo, Brazil)

150. Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1 (PubMed)

Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1 Pancreatico-duodenal tumors are the second most common endocrinopathy in multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of hormone excess and, in particular, the management of multiple endocrine neoplasia syndrome type 1

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2012 Clinics

151. Primary hyperparathyroidism in multiple endocrine neoplasia type 1: when to perform surgery? (PubMed)

Primary hyperparathyroidism in multiple endocrine neoplasia type 1: when to perform surgery? Primary hyperparathyroidism is a common endocrinological disorder. In rare circumstances, it is associated with familial syndromes, such as multiple endocrine neoplasia type 1. This syndrome is caused by a germline mutation in the multiple endocrine neoplasia type 1 gene encoding the tumor-suppressor protein menin. Usually, primary hyperparathyroidism is the initial clinical expression in carriers (...) of multiple endocrine neoplasia type 1 mutations, occurring in more than 90% of patients and appearing at a young age (20-25 years). Multiple endocrine neoplasia type 1/primary hyperparathyroidism is generally accompanied by multiglandular disease, clinically manifesting with hypercalcemia, although it can remain asymptomatic for a long time and consequently not always be recognized early. Surgery is the recommended treatment. The goal of this short review is to discuss the timing of surgery in patients

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2012 Clinics

152. Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy (PubMed)

Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy Usually, primary hyperparathyroidism is the first endocrinopathy to be diagnosed in patients with multiple endocrine neoplasia type 1, and is also the most common one. The timing of the surgery and strategy in multiple endocrine neoplasia type 1/hyperparathyroidism are still under debate. The aims of surgery are to: 1) correct hypercalcemia, thus preventing (...) observed in multiple endocrine neoplasia type 1 than in sporadic hyperparathyroidism. The recurrence rate is strongly influenced by: 1) the lack of a pre-operative multiple endocrine neoplasia type 1 diagnosis; 2) the surgeon's experience; 3) the timing of surgery; 4) the possibility of performing intra-operative confirmation (histologic examination, rapid parathyroid hormone assay) of the curative potential of the surgical procedure; and, 5) the surgical strategy. Persistent hyperparathyroidism seems

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2012 Clinics

153. Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center (PubMed)

Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center Most cases of sporadic primary hyperparathyroidism present disturbances in a single parathyroid gland and the surgery of choice is adenomectomy. Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical (...) multiglandular disease and it is surgically approached by either subtotal parathyroidectomy or total parathyroidectomy followed by parathyroid auto-implant to the forearm. In skilful hands, the efficacy of both approaches is similar and both should be complemented by prophylactic thymectomy. In a single academic center, 83 cases of hyperparathyroidism/ multiple endocrine neoplasia type 1 were operated on from 1987 to 2010 and our first surgical choice was total parathyroidectomy followed by parathyroid auto

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2012 Clinics

154. Multiple Endocrine Neoplasia Type 1

Multiple Endocrine Neoplasia Type 1 Multiple Endocrine Neoplasia Type 1 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 1 Multiple Endocrine Neoplasia Type 1 Aka: Multiple Endocrine Neoplasia Type 1 , Multiple Endocrine Neoplasia Type I , Werner's Syndrome , MEN I From Related Chapters II. Associated Conditions Familial (occurs in >80% of MEN I cases) Pancreatic islet tumors Zollinger-Ellison Syndrome Hypergastrinemia III. Prevention: Family Members of MEN I patients Consider annual lab screening starting at age 8 years annually Consider PTH Level annually or if is abnormal IV. References Images

2015 FP Notebook

155. Partial Pancreaticoduodenectomy Can Provide Cure for Duodenal Gastrinoma Associated With Multiple Endocrine Neoplasia Type 1. (PubMed)

Partial Pancreaticoduodenectomy Can Provide Cure for Duodenal Gastrinoma Associated With Multiple Endocrine Neoplasia Type 1. To evaluate the outcome of pancreaticoduodenectomy (PD) versus non-PD resections for the treatment of gastrinoma in multiple endocrine neoplasia type 1.Gastrinoma in MEN1 is considered a rarely curable disease and its management is highly controversial both for timing and extent of surgery.Clinical characteristics, complications and outcomes of 27 prospectively collected (...) MEN1 patients with biochemically proven gastrinoma, who underwent surgery, were analyzed with special regard to the gastrinoma type and the initial operative procedure.Twenty-two (81%) patients with gastrinoma in MEN1 had duodenal gastrinomas and 5 patients (19%) had pancreatic gastrinomas. At the time of diagnosis, 21 (77%) gastrinomas were malignant (18 duodenal, 3 pancreatic), but distant metastases were only present in 4 (15%) patients. Patients with pancreatic gastrinomas underwent either

2012 Annals of Surgery

156. Proliferation Rates of Multiple Endocrine Neoplasia Type 1 (MEN1)-Associated Tumors. (PubMed)

Proliferation Rates of Multiple Endocrine Neoplasia Type 1 (MEN1)-Associated Tumors. Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the combined occurrence of parathyroid and adrenocortical tumors, and neuroendocrine tumors (NETs) of the pancreas and pituitary. The pancreatic NETs are predominantly gastrinomas and insulinomas, and the pituitary NETs are mostly prolactinomas and somatotrophinomas. We postulated that the different types of pancreatic (...) and pituitary NETs may be partly due to differences in their proliferation rates, and we therefore assessed these in MEN1-associated tumors and gonadal tumors that developed in mice deleted for an Men1 allele (Men1(+/-)). To label proliferating cells in vivo, Men1(+/-) and wild-type (Men1(+/+)) mice were given 5-bromo-2-deoxyuridine (BrdU) in drinking water from 1-12 wk, and tissue sections were immunostained using anti-BrdU and hormone-specific antibodies. Proliferation in the tumors of Men1(+/-) mice

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2012 Endocrinology

157. Multiple Dose Study to Evaluate the Efficacy, Safety and Pharmacodynamics of REMD-477 in Subjects With Type 1 Diabetes Mellitus

Multiple Dose Study to Evaluate the Efficacy, Safety and Pharmacodynamics of REMD-477 in Subjects With Type 1 Diabetes Mellitus Multiple Dose Study to Evaluate the Efficacy, Safety and Pharmacodynamics of REMD-477 in Subjects With Type 1 Diabetes Mellitus - 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. Multiple Dose Study to Evaluate the Efficacy, Safety and Pharmacodynamics of REMD-477 in Subjects With Type 1 Diabetes Mellitus The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated by the U.S. Federal Government. of clinical studies and talk to your health care provider before participating. Read our

2017 Clinical Trials

158. Aggressive Medullary Thyroid Carcinoma in a 10-Year-Old Patient with Multiple Endocrine Neoplasia 2B due to the A883F Mutation. (PubMed)

Aggressive Medullary Thyroid Carcinoma in a 10-Year-Old Patient with Multiple Endocrine Neoplasia 2B due to the A883F Mutation. 25244518 2015 09 10 2015 01 10 1557-9077 25 1 2015 Jan Thyroid : official journal of the American Thyroid Association Thyroid Aggressive medullary thyroid carcinoma in a ten-year-old patient with multiple endocrine neoplasia 2B due to the A883F mutation. 139-40 10.1089/thy.2014.0177 Mathiesen Jes Sloth JS 1 Department of ENT Head & Neck Surgery, Odense University (...) Hospital , Odense C, Denmark . Stochholm Kirstine K Poulsen Per Loegstrup PL Vestergaard Else Marie EM Christiansen Peer P Vestergaard Peter P eng Case Reports Letter United States Thyroid 9104317 1050-7256 EC 2.7.10.1 Proto-Oncogene Proteins c-ret IM Carcinoma, Medullary genetics pathology Child Humans Male Multiple Endocrine Neoplasia Type 2b genetics pathology Mutation Proto-Oncogene Proteins c-ret genetics Thyroid Neoplasms genetics pathology 2014 9 23 6 0 2014 9 23 6 0 2015 9 12 6 0 ppublish

2014 Thyroid

159. Multiple Endocrine Neoplasia, Childhood

associated with RET germline mutations in the context of multiple endocrine neoplasia type 2 syndrome.[ ] Anaplastic carcinoma: Less than 1% of pediatric thyroid carcinomas are anaplastic carcinoma. Molecular Features Thyroid Carcinoma of Follicular Cells Thyroid tumorigenesis and progression of thyroid carcinomas of follicular cells (differentiated thyroid carcinoma, poorly-differentiated papillary thyroid carcinoma, and anaplastic thyroid carcinoma) are defined by a multistep process that results (...) or de novo gain-of-function mutation in the RET proto-oncogene associated with multiple endocrine neoplasia (MEN) type 2, either MEN2A or MEN2B, depending on the specific mutation.[ ] When occurring in patients with the MEN syndromes, thyroid cancer may be associated with the development of other types of malignant tumors. (Refer to the section of the PDQ summary on for more information.) Family history. For thyroid carcinomas of follicular cells, only 5% to 10% are familial cancers. Of those, most

2012 PDQ - NCI's Comprehensive Cancer Database

160. Use of Endocrine Therapy for Breast Cancer Risk Reduction

may be an alternative in women with intraepithelial neoplasia. Upper Age Limit for Endocrine Risk Reduction Therapy Women age 70 years or older should not be offered endocrine prevention unless their short-term risk is in the range of 1% or more per year (eg, atypical hyperplasia plus family history or LCIS), they are active, and they have a life expectancy of 10 or more years. Risk benefit will vary by age, medical circumstances, and the prevention drug. Longer Duration of Endocrine Therapy (...) at increased risk, the choice of endocrine therapy now includes anastrozole (1 mg/day) in addition to exemestane (25 mg/day), raloxifene (60 mg/day), or tamoxifen (20 mg/day). The decision regarding choice of endocrine therapy should take into consideration age, baseline comorbidities, and adverse effect profiles. Clinicians should not prescribe anastrozole, exemestane, or raloxifene for breast cancer risk reduction to premenopausal women. Tamoxifen 20 mg/day for 5 years is still considered standard

2019 American Society of Clinical Oncology Guidelines

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