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Multiple Endocrine Neoplasia Type 1

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161. Elacestrant (RAD1901), a selective estrogen receptor degrader (SERD), has anti-tumor activity in multiple ER+ breast cancer patient-derived xenograft models. Full Text available with Trip Pro

Elacestrant (RAD1901), a selective estrogen receptor degrader (SERD), has anti-tumor activity in multiple ER+ breast cancer patient-derived xenograft models. Purpose: Estrogen receptor-positive (ER+) breast cancers are typically treated with endocrine agents, and dependence on the ER pathway is often retained even after multiple rounds of antiestrogen therapy. Selective estrogen receptor degraders (SERD) are being developed as a strategy to more effectively target ER and exploit ER dependence (...) in these cancers, which includes inhibiting both wild-type and mutant forms of ER. The purpose of this study was to evaluate the efficacy of a novel orally bioavailable SERD, elacestrant (RAD1901), in preclinical models of ER+ breast cancer.Experimental Design: Elacestrant was evaluated as a single agent and in combination with palbociclib or everolimus in multiple ER+ breast cancer models, including several patient-derived xenograft models.Results: Elacestrant induces the degradation of ER, inhibits ER

2017 Clinical Cancer Research

162. Study of Axitinib for Reducing Extent of Venous Tumour Thrombus in Renal Cancer With Venous Invasion

in and Exported from the U.S.: No Keywords provided by Scottish Clinical Trials Research Unit: Neoadjuvant Venous Tumour Thrombus Axitinib Mayo Classification Nephrectomy Thrombectomy Additional relevant MeSH terms: Layout table for MeSH terms Carcinoma Carcinoma, Renal Cell Thrombosis Neoplasms, Glandular and Epithelial Neoplasms by Histologic Type Neoplasms Adenocarcinoma Kidney Neoplasms Urologic Neoplasms Urogenital Neoplasms Neoplasms by Site Kidney Diseases Urologic Diseases Embolism and Thrombosis (...) of enrolment. The presence of active second malignancy. Patients will be eligible if they have adequately treated basal cell carcinoma, squamous cell skin cancer, in situ cervical cancer, stable prostate cancer or if treated with curative intent for any other cancer with no evidence of disease for 2 years. Patients with prostate cancer will be permitted entry if not receiving treatment and prostrate-specific antigen (PSA) is not rising. Women who are pregnant or are breastfeeding. Female patients must

2018 Clinical Trials

163. Testicle, Malignant Tumors

lesions. The embryonal cell tumor is the classic pure-cell, nonseminomatous tumor and is relatively uncommon (20%). Choriocarcinoma represents the most lethal but least common (1%) nonseminomatous type. The depiction here is classic for a seminoma. Testicular malignancies appear as a hypoechoic mass in the vast majority of cases. This is another seminoma. On sonograms, a seminoma is often more homogeneous than nonseminomatous cancers. Yolk sac tumors, which are also known as endodermal sinus tumors (...) is not a current recommendation, because patients with stage 1 or 2 (2A or 2B) testicular cancer have a long-term, disease-free survival rate greater than 95%; in patients with bulky retroperitoneal nodes and/or pulmonary or visceral metastasis, this rate is 60-80%. Preferred examination Ultrasonography is the standard imaging technique used to identify testicular carcinoma. It has a high sensitivity, but it must be combined with physical examination to achieve the best specificity. More than 95% of testicular

2014 eMedicine Radiology

164. Malignant Tumors of the Sinuses

lymphoma (SN-ETNKL). The study, which involved 1054 patients with SN-DLBCL and 328 patients with SN-ETNKL, found that when patients were matched for age, cancer stage, and treatment modality, disease-specific survival rates for SN-DLBCL at 1, 5, and 10 years were 94.4%, 72.8%, and 46.8%, respectively, compared with 77.6%, 38.4%, and 13.9%, respectively, for patients with SN-ETNKL. [ ] Salivary-type neoplasms Pleomorphic adenomas, mucoepidermoid carcinoma, and other salivary gland neoplasms may arise (...) and often confusing group of sinonasal malignancies (SNM) including esthesioneuroblastoma (ENB), sinonasal undifferentiated carcinoma (SNUC), neuroendocrine carcinoma (NEC), and small cell carcinoma (SmCC). Although important histologic differences exist between these tumors, most experts agree that the largest distinction should be made between ENB and non-ENB types because of the much more favorable clinical outcome of ENB compared with the other 3. [ ] Part of the confusion comes from the multiple

2014 eMedicine Surgery

165. A rare presentation of multiple endocrine neoplasia (MEN) type 2A syndrome Full Text available with Trip Pro

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.

2015 Annals of Medicine and Surgery

166. Management of the Parathyroid Glands During Preventive Thyroidectomy in Patients With Multiple Endocrine Neoplasia Type 2. Full Text available with Trip Pro

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

2015 Annals of Surgery

167. Therapeutic Effectiveness of Screening for Multiple Endocrine Neoplasia Type 2A. Full Text available with Trip Pro

Therapeutic Effectiveness of Screening for Multiple Endocrine Neoplasia Type 2A. Although technological progress revolutionized detection of genetic predisposition to medullary thyroid cancer (MTC), carriers of mutations of disparate risks may not have benefitted alike from screening.This investigation aimed at assessing the achievements of screening for multiple endocrine neoplasia type 2A (MEN 2A) in Germany and identifying current challenges.This was a retrospective analysis comprising 455

2015 Journal of Clinical Endocrinology and Metabolism

168. Postoperative Complications After Prophylactic Thyroidectomy for Very Young Patients With Multiple Endocrine Neoplasia Type 2: Retrospective Cohort Analysis. Full Text available with Trip Pro

Postoperative Complications After Prophylactic Thyroidectomy for Very Young Patients With Multiple Endocrine Neoplasia Type 2: Retrospective Cohort Analysis. The aim of this study was to investigate whether younger age at surgery is associated with the increased incidence of postoperative complications after prophylactic thyroidectomy in pediatric patients with multiple endocrine neoplasia (MEN) 2. The shift toward earlier thyroidectomy has resulted in significantly less medullary thyroid (...) carcinoma (MTC)-related morbidity and mortality. However, very young pediatric patients might have a higher morbidity rate compared with older patients. Hardly any literature exists on complications in the very young. A retrospective single-center analysis was performed on the outcomes of MEN2 patients undergoing a prophylactic total thyroidectomy at the age of 17 or younger. Forty-one MEN2A and 3 MEN2B patients with thyroidectomy after January 1993 and at least 6 months of follow-up were included

2015 Medicine

169. A rare case of juvenile hypertension: coexistence of type 2 multiple endocrine neoplasia -related bilateral pheochromocytoma and reninoma in a young patient with ACE gene polymorphism. Full Text available with Trip Pro

A rare case of juvenile hypertension: coexistence of type 2 multiple endocrine neoplasia -related bilateral pheochromocytoma and reninoma in a young patient with ACE gene polymorphism. Pheochromocytoma and reninoma represent two rare diseases causing hypertension. We here reported a rare case of association between type 2 multiple endocrine neoplasia related bilateral pheochromocytoma and reninoma. Moreover, polymorphism of ACE gene, which is known to be related to an increase of cardiovascular (...) risk, has been found in the same patient.A 24 year old Caucasian man came to our attention for severe hypertension, resistant to anti-hypertensive polytherapy. At the age of twenty he had undergone total thyroidectomy with lymphadenectomy for medullary carcinoma. Genetic testing showed a RET mutation of codon 918 (exon 16) not documented in other family members. During the follow-up, a progressive increase of urinary metanephrines and catecholamines was recorded. Our evaluation confirmed

2015 BMC Endocrine Disorders

170. Corneal xanthogranuloma in association with multiple endocrine neoplasia 1: A clinicopathologic case report and review of the literature Full Text available with Trip Pro

an extensive right eye corneal lesion in a 43-year old male which showed the typical histopathological feature of JXG and in association with multiple endocrine neoplasia (type 1). Similar cases in the English-language literature have been also reviewed. (...) Corneal xanthogranuloma in association with multiple endocrine neoplasia 1: A clinicopathologic case report and review of the literature Juvenile xanthogranuloma (JXG) is a benign inflammatory condition of uncertain pathogenesis. It is characterized by skin and ocular involvement - typically in the iris - in children. It has been reported in older age groups and has been also observed to involve other ocular structures such as the cornea and conjunctiva. In this case report, we are presenting

2015 Saudi Journal of Ophthalmology

171. Multiple endocrine neoplasia syndromes 1 and 2: manifestations and management in childhood and adolescence. (Abstract)

Multiple endocrine neoplasia syndromes 1 and 2: manifestations and management in childhood and adolescence. The identification of the genetic causes of the multiple endocrine neoplasia (MEN) syndromes 1 and 2, and associated genotype-phenotype relationships, has revolutionised the clinical care of affected patients. A genetic diagnosis can be made during infancy and careful clinical surveillance, coupled with early intervention, has the potential to improve both morbidity and mortality

2015 Archives of Disease in Childhood

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

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 (...) 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

2014 Thyroid

173. Bilateral pheochromocytoma with ganglioneuroma component associated with multiple neuroendocrine neoplasia type 2A: a case report Full Text available with Trip Pro

Bilateral pheochromocytoma with ganglioneuroma component associated with multiple neuroendocrine neoplasia type 2A: a case report Composite pheochromocytoma/paragangliomas are very rare tumors composed of ordinary pheochromocytoma paragangliomas associated with neurogenic tumors. Several hereditary susceptibility disorders are known to be associated with pheochromocytoma/paragangliomas such as multiple endocrine neoplasia type 2 (2A or B). To the best of our knowledge, only four cases (...) of composite pheochromocytoma/paragangliomas associated with multiple endocrine neoplasia type 2 have been reported.A 40-year-old Arabic woman presented with headache, palpitations, paroxysmal hypertension, and weight loss, which she had had for the last 3 years. She had a familial history of diabetes and multiple endocrine neoplasia type 2. A radiological examination revealed thyroid lesions and bilateral adrenal medulla tumors. Our patient had undergone bilateral adrenalectomy, total thyroidectomy

2017 Journal of medical case reports

174. Surgical approach to medullary thyroid carcinoma associated with multiple endocrine neoplasia type 2 Full Text available with Trip Pro

Surgical approach to medullary thyroid carcinoma associated with multiple endocrine neoplasia type 2 We briefly review the surgical approaches to medullary thyroid carcinoma associated with multiple endocrine neoplasia type 2 (medullary thyroid carcinoma/multiple endocrine neoplasia type 2). The recommended surgical approaches are usually based on the age of the affected carrier/patient, tumor staging and the specific rearranged during transfection codon mutation. We have focused mainly (...) on young children with no apparent disease who are carrying a germline rearranged during transfection mutation. Successful management of medullary thyroid carcinoma in these cases depends on early diagnosis and treatment. Total thyroidectomy should be performed before 6 months of age in infants carrying the rearranged during transfection 918 codon mutation, by the age of 3 years in rearranged during transfection 634 mutation carriers, at 5 years of age in carriers with level 3 risk rearranged during

2012 Clinics

175. Long-term Follow-up of MEN1 Patients Who Do Not Have Initial Surgery for Small ≤2 cm Nonfunctioning Pancreatic Neuroendocrine Tumors, an AFCE and GTE Study: Association Francophone de Chirurgie Endocrinienne & Groupe d'Etude des Tumeurs Endocrines. Full Text available with Trip Pro

Long-term Follow-up of MEN1 Patients Who Do Not Have Initial Surgery for Small ≤2 cm Nonfunctioning Pancreatic Neuroendocrine Tumors, an AFCE and GTE Study: Association Francophone de Chirurgie Endocrinienne & Groupe d'Etude des Tumeurs Endocrines. To report long-term follow-up of patients with multiple endocrine neoplasia type 1 (MEN1) and nonfunctioning pancreatic neuroendocrine tumors (NF-PET).Pancreaticoduodenal tumors occur in almost all patients with MEN1 and are a major cause of death (...) unrelated to MEN1. Twenty-eight patients had stable disease and 16 showed significant progression of pancreaticoduodenal involvement, indicated by increase in size or number of tumors, development of a hypersecretion syndrome, need for surgery (7 patients), and death from metastatic NF-PET (1 patient). The mean event-free survival was 13.9 ± 1.1 years after NF-PET diagnosis. At last follow-up, none of the living patients who had undergone surgery or follow-up had evidence of metastases on imaging

2017 Annals of Surgery

176. The histone methyltransferase EZH2, an oncogene common to benign and malignant parathyroid tumors. Full Text available with Trip Pro

The histone methyltransferase EZH2, an oncogene common to benign and malignant parathyroid tumors. Primary hyperparathyroidism (pHPT) resulting from parathyroid tumors is a common endocrine disorder with incompletely understood etiology. In renal failure, secondary hyperparathyroidism (sHPT) occurs with multiple tumor development as a result of calcium and vitamin D regulatory disturbance. The aim of this study was to investigate a potential role of the histone 3 lysine 27 methyltransferase (...) EZH2 in parathyroid tumorigenesis. Parathyroid tumors from patients with pHPT included adenomas and carcinomas. Hyperplastic parathyroid glands from patients with HPT secondary to uremia and normal parathyroid tissue specimens were included in this study. Quantitative RT-PCR, western blotting, bisulfite pyrosequencing, colony formation assay, and RNA interference were used. EZH2 was overexpressed in a subset of the benign and in all malignant parathyroid tumors as determined by quantitative RT-PCR

2013 Endocrine-Related Cancer

177. 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

178. 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

179. 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

180. 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

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