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Multiple Endocrine Neoplasia Type 1

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181. Newer Drugs for Type 2 Diabetes: An Emerging Adjunctive Therapy to Insulin for Type 1 Diabetes?

Newer Drugs for Type 2 Diabetes: An Emerging Adjunctive Therapy to Insulin for Type 1 Diabetes? Newer Drugs for Type 2 Diabetes: An Emerging Adjunctive Therapy to Insulin for Type 1 Diabetes? | CADTH.ca CADTH Document Viewer Newer Drugs for Type 2 Diabetes: An Emerging Adjunctive Therapy to Insulin for Type 1 Diabetes? Table of Contents Search this document Newer Drugs for Type 2 Diabetes: An Emerging Adjunctive Therapy to Insulin for Type 1 Diabetes? January 2018 Summary There are two major (...) categories of diabetes. Type 1 diabetes (T1D) is characterized by severe impairment or an absolute deficiency of insulin due to autoimmune destruction of pancreatic cells. Type 2 diabetes (T2D) is characterized by a combination of insulin resistance and decreased insulin secretion. Insulin is the mainstay of treatment for T1D. However, it may be difficult for some patients to reach target glycated hemoglobin (A1C) levels on insulin monotherapy. In some cases, hypoglycemia, excessive glucose fluctuations

2018 CADTH - Issues in Emerging Health Technologies

182. Endocrine neoplasms in familial syndromes of hyperparathyroidism. Full Text available with Trip Pro

Endocrine neoplasms in familial syndromes of hyperparathyroidism. Familial syndromes of hyperparathyroidism, including multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), and the hyperparathyroidism-jaw tumor (HPT-JT), comprise 2-5% of primary hyperparathyroidism cases. Familial syndromes of hyperparathyroidism are also associated with a range of endocrine and nonendocrine tumors, including potential malignancies. Complications of the associated neoplasms (...) are the major causes of morbidities and mortalities in these familial syndromes, e.g., parathyroid carcinoma in HPT-JT syndrome; thymic, bronchial, and enteropancreatic neuroendocrine tumors in MEN1; and medullary thyroid cancer and pheochromocytoma in MEN2A. Because of the different underlying mechanisms of neoplasia, these familial tumors may have different characteristics compared with their sporadic counterparts. Large-scale clinical trials are frequently lacking due to the rarity of these diseases

2016 Endocrine-Related Cancer

183. A randomized, prospective trial of operative treatments for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. (Abstract)

A randomized, prospective trial of operative treatments for hyperparathyroidism in patients with multiple endocrine neoplasia type 1. Hyperparathyroidism (HPT) in multiple endocrine neoplasia (MEN) type 1 is associated with multiglandular parathyroid disease. Previous retrospective studies comparing subtotal parathyroidectomy (SP) and total parathyroidectomy with autotransplantation (TP/AT) have not established clearly better outcomes with either procedure.Patients were assigned randomly (...) %) overall. The rate of permanent hypoparathyroidism was 12% in the SP group (2/17) and 7% in the TP/AT group (1/15). A second operation was performed in 4 of 17 patients initially treated with SP (24%), compared with 1 of 15 patients undergoing TP/AT (7%; P = .34).This randomized trial of SP and TP/AT in patients with MEN 1 failed to show any difference in outcomes when comparing results of SP versus TP/AT. Both procedures are associated with acceptable results, but SP may have advantages

2014 Surgery Controlled trial quality: uncertain

184. Genetic Analysis of Multiple Endocrine Neoplasia Type 1 (MEN1) Leads to Misdiagnosis of an Extremely Rare Presentation of Intrasellar Cavernous Hemangioma as MEN1 Full Text available with Trip Pro

Genetic Analysis of Multiple Endocrine Neoplasia Type 1 (MEN1) Leads to Misdiagnosis of an Extremely Rare Presentation of Intrasellar Cavernous Hemangioma as MEN1 Multiple endocrine neoplasia type 1 (MEN1) is a rare inherited disorder characterized by the simultaneous occurrence of endocrine tumors in target tissues (mainly the pituitary, endocrine pancreas, and parathyroid glands). MEN1 is caused by mutations in the MEN1 gene, which functions as a tumor suppressor and consists of one (...) untranslated exon and nine exons encoding the menin protein. This condition is usually suspected when we encounter patients diagnosed with tumors in multiple endocrine organs, as mentioned above.A 65-year-old woman who underwent surgery for a pancreatic tumor (serous cystadenoma) 5 years previously was referred to our hospital due to neurologic symptoms of diplopia and left ptosis. Brain magnetic resonance imaging revealed a 3.4-cm lesion originating from the cavernous sinus wall and extending

2014 Endocrinology and Metabolism

185. Genetic and Epigenetic Analysis in Korean Patients with Multiple Endocrine Neoplasia Type 1 Full Text available with Trip Pro

Genetic and Epigenetic Analysis in Korean Patients with Multiple Endocrine Neoplasia Type 1 Multiple endocrine neoplasia type 1 (MEN1) is a familial syndrome characterized by the parathyroid, pancreas and pituitary tumors. Parathyroid tumors are the most common clinical manifestations, occurring in more than 90% of MEN1 patients. Heterozygous germline mutations of the MENIN gene underlie the tumorigenesis in MEN1 and epigenetic alterations along with germline mutations may contribute (...) to tumorigenesis. Here, we investigated the associations between genotype and phenotype in Korean MEN1 patients.We analyzed medical records from 14 unrelated MEN1 patients who had newly confirmed MENIN germline mutations, together with 14 previous reports in Korea. Aberrant DNA methylations were also examined in MEN1-related parathyroid tumors using the Infinium HumanMethylation 450 BeadChip.Total 28 germline mutations of MENIN were relatively highly concentrated in exons 7 and 8 compared to previous reports

2014 Endocrinology and Metabolism

186. Safety and Efficacy of Percutaneous Parathyroid Ethanol Ablation in Patients with Recurrent Primary Hyperparathyroidism and Multiple Endocrine Neoplasia Type 1. Full Text available with Trip Pro

Safety and Efficacy of Percutaneous Parathyroid Ethanol Ablation in Patients with Recurrent Primary Hyperparathyroidism and Multiple Endocrine Neoplasia Type 1. The most common feature of multiple endocrine neoplasia type 1 (MEN1) is primary hyperparathyroidism (PHP), which occurs in approximately 95% of MEN1 patients. Approximately 40-60% of patients with MEN1 develop recurrent hypercalcemia within 10-12 years after their initial parathyroid surgery and the successful management of recurrent

2014 Journal of Clinical Endocrinology and Metabolism

187. Preoperative multiple endocrine neoplasia type 1 diagnosis improves the surgical outcomes of pediatric patients with primary hyperparathyroidism. (Abstract)

Preoperative multiple endocrine neoplasia type 1 diagnosis improves the surgical outcomes of pediatric patients with primary hyperparathyroidism. Primary hyperparathyroidism (PHPT) is uncommon in children. The surgical management of PHPT in children has evolved over the past two decades.A retrospective study of patients who underwent parathyroidectomy for PHPT diagnosed at age < 18 years and managed at a tertiary referral center for endocrine and familial disorders.Thirty-eight patients met (...) eligibility criteria (1981-2012). Median age at PHPT diagnosis was 15 years. Two-thirds of patients were symptomatic (68%, n=26), most commonly from nephrolithiasis. Twenty-six (68%) patients underwent a standard cervical exploration while 32% underwent a focused unilateral parathyroidectomy. Multiple endocrine neoplasia type 1 (MEN1) was diagnosed preoperatively in 22/26 patients. Patients with a preoperative diagnosis of MEN1 were more likely to undergo a complete initial operation (≥ 3 gland

2014 Journal of Pediatric Surgery

188. SSO/ASTRO/ASCO Consensus Guideline on Margins for Breast Conserving Surgery (BCS) with Whole Breast Irradiation in Ductal Carcinoma in Situ (DCIS) Full Text available with Trip Pro

rates of ipsilateral breast tumor recurrence (IBTR), even among patients with smaller, non–high-grade DCISs. x [1] Correa, C, McGale, P, Taylor, C et al. Overview of the randomized trials of radiotherapy in ductal carcinoma in situ of the breast. J Natl Cancer Inst Monogr . 2010 ; 2010 : 162–177 | | | , x [4] McCormick, B, Winter, K, Hudis, C et al. RTOG 9804: A prospective randomized trial for good-risk ductal carcinoma in situ comparing radiotherapy with observation. J Clin Oncol . 2015 ; 33 : 709 (...) 2 mm. Introduction Breast-conserving therapy (BCT), defined as surgical excision of the primary tumor with a margin of surrounding normal tissue followed by whole-breast irradiation (WBRT), results in long-term cause-specific survival rates of greater than 95% for women with ductal carcinoma in situ (DCIS), as demonstrated in both randomized trials x [1] Correa, C, McGale, P, Taylor, C et al. Overview of the randomized trials of radiotherapy in ductal carcinoma in situ of the breast. J Natl

2019 American Society for Radiation Oncology

189. eUpdate: Hepatocellular Carcinoma Algorithms

Testicular Germ Cell CancerCancer of the Prostate • Bladder Cancer • Renal Cell Carcinoma • Penile Carcinoma • Testicular Seminoma and Non-Seminoma Gynaecological Cancers Cervical cancer • Endometrial cancer • Gestational trophoblastic disease • Newly diagnosed and relapsed epithelial ovarian carcinoma • Non-epithelial ovarian cancer • Ovarian Cancer Haematological Malignancies Waldenstrom's macroglobulinaemia • Chronic myeloid leukaemia • Newly diagnosed and relapsed mantle cell lymphoma • Multiple (...) marginal zone lymphoma of MALT type Head and Neck Cancers Nasopharyngeal Cancer • Squamous-Cell Carcinoma of the Head and Neck Hereditary Syndromes Prevention and Screening in BRCA Mutation Carriers and Other Breast/Ovarian Hereditary Cancer Syndromes Lung and Chest Tumours Early and locally advanced non-small-cell lung cancer • Metastatic non-small-cell lung cancer • Thymic epithelial tumoursMalignant pleural mesothelioma • Small-cell lung cancer Melanoma Cutaneous Melanoma Neuro-Oncology EANO

2020 European Society for Medical Oncology

190. eUpdate: Hepatocellular Carcinoma Treatment Recommendations

in adult patients • Gastric marginal zone lymphoma of MALT type Head and Neck Cancers Nasopharyngeal Cancer • Squamous-Cell Carcinoma of the Head and Neck Hereditary Syndromes Prevention and Screening in BRCA Mutation Carriers and Other Breast/Ovarian Hereditary Cancer Syndromes Lung and Chest Tumours Early and locally advanced non-small-cell lung cancer • Metastatic non-small-cell lung cancer • Thymic epithelial tumoursMalignant pleural mesothelioma • Small-cell lung cancer Melanoma Cutaneous (...) carcinoma Genitourinary Cancers Testicular Germ Cell CancerCancer of the Prostate • Bladder Cancer • Renal Cell Carcinoma • Penile Carcinoma • Testicular Seminoma and Non-Seminoma Gynaecological Cancers Cervical cancer • Endometrial cancer • Gestational trophoblastic disease • Newly diagnosed and relapsed epithelial ovarian carcinoma • Non-epithelial ovarian cancer • Ovarian Cancer Haematological Malignancies Waldenstrom's macroglobulinaemia • Chronic myeloid leukaemia • Newly diagnosed and relapsed

2020 European Society for Medical Oncology

191. Consensus guideline on margins for breast-conserving surgery with whole-breast irradiation in ductal carcinoma in situ Full Text available with Trip Pro

,FentimanIS,etal.Radiotherapyinbreast-conserving treatment for ductal carcinoma in situ: First results of the EORTC randomised phase III trial 10853: EORTC Breast Cancer Cooperative Group and EORTC Radiotherapy Group. Lancet. 2000;355(9203): 528-533. 7. Wapnir IL, Dignam JJ, Fisher B, et al. Long-term outcomes of invasive ipsilateral breast tumor recurrences after lumpectomy in NSABP B-17 and B-24 randomized clinical trials for DCIS. J Natl Cancer Inst. 2011;103(6):478-488. 8. Emdin SO, Granstrand B (...) -yearupdateofaprospective studyofwideexcisionaloneforsmalllow-orintermediate-gradeductal carcinoma in situ (DCIS). Breast Cancer Res Treat. 2014;143(2): 343-350. 26. CuzickJ,SestakI,PinderSE,etal.Effectoftamoxifenandradiotherapy inwomenwithlocallyexcisedductalcarcinomainsitu:Long-termresults fromtheUK/ANZDCIStrial.Lancet Oncol.2011;12(1):21-29. 27. Bijker N, Meijnen P, Peterse JL, et al. Breast-conserving treatment with or without radiotherapy in ductal carcinoma-in-situ: Ten-year results of European Organisation

2019 American Society for Radiation Oncology

192. Abemaciclib in combination with fulvestrant for advanced or metastatic breast cancer after prior endocrine therapy

half of people affected, it remains a serious and life threatening disease. Treatment does not work as well for patients with the ABC type compared to patients with other DLBCL types who receive standard treatment. February 2019 Acalabrutinib is a novel oral anti‐cancer drug in clinical development for people with chronic lymphocytic leukaemia (CLL) who have not received any previous treatment. CLL is a type of cancer in which too many white blood cells are produced. As these cells develop (...) Acalabrutinib is a novel oral anti-cancer drug in clinical development for people with relapsed or refractory (R/R) chronic lymphocytic leukaemia (CLL) who have previously been treated (second line or greater). CLL is a type of cancer in which too many white blood cells are produced. As these cells develop abnormally, they are unable to function and fight infection and stop the production of healthy blood cells. The disease is chronic and develops slowly. R/R CLL means the cancer has come back after

2017 NIHR Innovation Observatory

193. Successful cisplatin-etoposide chemotherapy-based treatment of a primary small cell neuroendocrine carcinoma of the tonsil with multiple metastases: a case report Full Text available with Trip Pro

Successful cisplatin-etoposide chemotherapy-based treatment of a primary small cell neuroendocrine carcinoma of the tonsil with multiple metastases: a case report Extrapulmonary small cell neuroendocrine carcinoma (SNEC) is an extremely rare and highly malignant tumor with a poor prognosis. Multiple metastases of SNEC are even more rare, and patients with locally advanced and metastatic disease generally face a poor outcome. To date, only a few cases of SNEC have been reported. Here, we (...) biopsy. She was additionally sent for a positron emission tomography scan to evaluate small metastases in the left cervical lymph node, right lung, multiple mediastinal lymph nodes, and the fourth lumbar (L4) vertebra body metastases. Histopathological examination of the SNEC confirmed a nested, typical endocrine appearance with small round cells containing ovoid-shaped nuclei and high mitotic activity. Immunohistochemically, the tumor cells were positive for cytokeratin 8/18+, synaptophysin+, CD56

2018 OncoTargets and therapy

194. Pleuropulmonary Blastoma, Childhood

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 (...) 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

2012 PDQ - NCI's Comprehensive Cancer Database

195. The early diagnosis of medullary carcinoma of the thyroid gland in patients with multiple endocrine neoplasia type II. Full Text available with Trip Pro

The early diagnosis of medullary carcinoma of the thyroid gland in patients with multiple endocrine neoplasia type II. Pentagastrin is a potent stimulator of thyrocalcitonin secretion from "C" cells. Since medulllary carcinoma of the thyroid gland (MCT) is a tumor composed of "C" cells, pentagastrin was used to screen a large kindred with multiple endocrine neoplasia type II (MCT, pheochromocytoma (s), and parathyroid hyperplasia). Four children with no thyroid abnormalities evident on physical (...) elevations in thyrocalcitonin secretion. This test has great diagnostic utility, especially in screening patients with multiple endocrine neoplasia type II.

1975 Annals of Surgery

196. Vandetanib (medullary thyroid carcinoma in children and adolescents from the age of 5) ? Benefit assessment according to § 35a Social Code Book V

endocrine neoplasia type 2B associated medullary thyroid carcinoma. Clin Cancer Res 2013; 19(15): 4239-4248. 9. AstraZeneca. Phase I/II trial of vandetanib (ZD6474, ZACTIMA) in children and adolescents with hereditary medullary thyroid carcinoma: study IRUSZACT0098; clinical study report [unpublished]. 2013. Extract of dossier assessment A17-01 Version 1.1 Vandetanib (thyroid cancer) 1 June 2017 Institute for Quality and Efficiency in Health Care (IQWiG) - 9 - 10. National Cancer Institute. Vandetanib (...) ? Carolin Weigel Keywords: vandetanib, thyroid neoplasms, child, benefit assessment 2 Due to legal data protection regulations, employees have the right not to be named. Extract of dossier assessment A17-01 Version 1.1 Vandetanib (thyroid cancer) 1 June 2017 Institute for Quality and Efficiency in Health Care (IQWiG) - iii - Table of contents Page List of tables iv List of abbreviations v 2 Benefit assessment 1 2.1 Executive summary of the benefit assessment 1 2.2 Research question 3 2.3 Information

2017 Institute for Quality and Efficiency in Healthcare (IQWiG)

197. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumours Full Text available with Trip Pro

is usually higher than in series including all patients presenting with an adrenal mass. There is, however, clear evidence that the vast majority of adrenal incidentalomas are benign adrenocortical adenomas. Table 1 Adrenal incidentalomas – frequency of the different underlying tumor types (adapted according ( )). Due to the nature of these studies, a selection bias is very probable (the populations studied not reflecting a random sample of all patients with an adrenal incidentalomas) and most likely (...) tumors), or histology after surgery or biopsy (for benign or malignant adrenal tumors) •Reporting 2×2 contingency table data or at least two indices of diagnostic accuracy (sensitivity, specificity, negative or positive predictive value) and disease prevalence •5496 abstracts b •525 potentially relevant articles •37 studies included in systematic review, 18 in meta-analysis •Major reasons for exclusion of articles were lack of test accuracy data, inadequate or unclear reference standard

2016 European Society of Endocrinology

198. Multiple Endocrine Neoplasia Type 1 (Diagnosis)

Tumors in Multiple Endocrine Neoplasia Type 1: The Mayo Clinic Experience from 1977 to 2013. Horm Cancer . 2015 Jun 13. . Bartsch DK, Albers MB, Lopez CL, Apitzsch JC, Walthers EM, Fink L, et al. Bronchopulmonary Neuroendocrine Neoplasms and Their Precursor Lesions in Multiple Endocrine Neoplasia Type 1. Neuroendocrinology . 2015 Jun 18. . Sakurai A, Hashizume K, Fukushima Y. Facial angiofibroma as an initial manifestation in multiple endocrine neoplasia type 1. Intern Med . 2008. 47(11):1067-8 (...) proliferation control of a menin in-frame deletion mutant associated with multiple endocrine neoplasia type 1 (MEN1). J Biol Chem . 2012 Jan 24. . Pack S, Turner ML, Zhuang Z, et al. Cutaneous tumors in patients with multiple endocrine neoplasia type 1 show allelic deletion of the MEN1 gene. J Invest Dermatol . 1998 Apr. 110(4):438-40. . Vortmeyer AO, Boni R, Pack SD, Darling TN, Zhuang Z. Perivascular cells harboring multiple endocrine neoplasia type 1 alterations are neoplastic cells in angiofibromas

2014 eMedicine.com

199. Multiple Endocrine Neoplasia Type 1 (Overview)

Tumors in Multiple Endocrine Neoplasia Type 1: The Mayo Clinic Experience from 1977 to 2013. Horm Cancer . 2015 Jun 13. . Bartsch DK, Albers MB, Lopez CL, Apitzsch JC, Walthers EM, Fink L, et al. Bronchopulmonary Neuroendocrine Neoplasms and Their Precursor Lesions in Multiple Endocrine Neoplasia Type 1. Neuroendocrinology . 2015 Jun 18. . Sakurai A, Hashizume K, Fukushima Y. Facial angiofibroma as an initial manifestation in multiple endocrine neoplasia type 1. Intern Med . 2008. 47(11):1067-8 (...) proliferation control of a menin in-frame deletion mutant associated with multiple endocrine neoplasia type 1 (MEN1). J Biol Chem . 2012 Jan 24. . Pack S, Turner ML, Zhuang Z, et al. Cutaneous tumors in patients with multiple endocrine neoplasia type 1 show allelic deletion of the MEN1 gene. J Invest Dermatol . 1998 Apr. 110(4):438-40. . Vortmeyer AO, Boni R, Pack SD, Darling TN, Zhuang Z. Perivascular cells harboring multiple endocrine neoplasia type 1 alterations are neoplastic cells in angiofibromas

2014 eMedicine.com

200. Multiple Endocrine Neoplasia Type 1 (Treatment)

. . Pack S, Turner ML, Zhuang Z, et al. Cutaneous tumors in patients with multiple endocrine neoplasia type 1 show allelic deletion of the MEN1 gene. J Invest Dermatol . 1998 Apr. 110(4):438-40. . Vortmeyer AO, Boni R, Pack SD, Darling TN, Zhuang Z. Perivascular cells harboring multiple endocrine neoplasia type 1 alterations are neoplastic cells in angiofibromas. Cancer Res . 1999 Jan 15. 59(2):274-8. . Rusconi D, Valtorta E, Rodeschini O, Giardino D, Lorenzo I, Predieri B, et al. Combined (...) man with recurrent nephrolithiasis, fatigue, and vision loss. J Am Acad Dermatol . 2009 Aug. 61(2):319-22. . . Asgharian B, Turner ML, Gibril F, Entsuah LK, Serrano J, Jensen RT. Cutaneous tumors in patients with multiple endocrine neoplasm type 1 (MEN1) and gastrinomas: prospective study of frequency and development of criteria with high sensitivity and specificity for MEN1. J Clin Endocrinol Metab . 2004 Nov. 89(11):5328-36. . Concolino P, Costella A, Capoluongo E. Multiple endocrine neoplasia

2014 eMedicine.com

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