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Multiple Endocrine Neoplasia Type 1

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161. An Open-Label, Multicenter, Phase 1/2 Study of Poly(ADP-Ribose) Polymerase (PARP) Inhibitor E7449 as Single Agent in Subjects With Advanced Solid Tumors or With B-cell Malignancies and in Combination With Temozolomide (TMZ) or With Carboplatin and Paclita

Melanoma Ovarian Neoplasms Carcinoma, Ovarian Epithelial Triple Negative Breast Neoplasms Leukemia, Lymphocytic, Chronic, B-Cell Neuroendocrine Tumors Neuroectodermal Tumors Neoplasms, Germ Cell and Embryonal Neoplasms by Histologic Type Neoplasms, Nerve Tissue Nevi and Melanomas Endocrine Gland Neoplasms Neoplasms by Site Ovarian Diseases Adnexal Diseases Genital Diseases, Female Genital Neoplasms, Female Urogenital Neoplasms Endocrine System Diseases Gonadal Disorders Carcinoma Neoplasms, Glandular (...) Intervention/treatment Phase Malignant Solid Tumour Ovarian Cancer Triple Negative Breast Cancer Advanced Melanoma B-cell Malignancy, Low-grade Drug: E7449 alone Drug: E7449 plus TMZ Drug: E7449 plus carboplatin and paclitaxel Phase 1 Phase 2 Study Design Go to Layout table for study information Study Type : Interventional (Clinical Trial) Actual Enrollment : 41 participants Allocation: Non-Randomized Intervention Model: Single Group Assignment Masking: None (Open Label) Primary Purpose: Treatment Official

2012 Clinical Trials

162. 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

163. Listeria monocytogenes vaccine for unresectable malignant plural mesothelioma

as these outputs are produced as required for our stakeholders. > > > Listeria monocytogenes vaccine for unresectable malignant plural mesothelioma Listeria monocytogenes vaccine for unresectable malignant plural mesothelioma September 2017 Malignant pleural mesothelioma is a type of cancer of the lungs caused mostly by exposure to asbestos. When asbestos fibres are inhaled, they can cause tumours to grow in the pleura, the thin membrane of cells (mesothelium) that line the lungs and chest wall. The disease (...) known as R-CHOP is in clinical development for newly diagnosed, previously untreated adult patients with diffuse large B-cell lymphoma (DLBCL) of the subtype known as activated B-cells (ABC) type. DLBCL is a cancer affecting a type of white blood cells called lymphocytes or B-cells. It is the most common form of non-Hodgkin lymphoma among adults. DLBCL is an aggressive cancer and although it can be cured in more than half of people affected, it remains a serious and life threatening disease

2017 NIHR Innovation Observatory

164. Quality Improvement Guidelines for Transarterial Chemoembolization and Embolization of Hepatic Malignancy

—which build on prior versions of this document—have thus been developed for use in QI programs assessing transarterial chemoembolization and embolization outcomes in clinical practice. CLINICAL BACKGROUND ON LIVER TUMORS Hepatocellular Carcinoma Hepatocellular carcinoma (HCC) accounts for 85%–90% of all primary liver cancers and is a signi?cant cause of morbidity and mortality worldwide (1). The incidence of HCC continues to increase both inter- nationally and in the United States, currently (...) and include liver-dominant disease, preserved hepatic function, and retained performance status (31). Other Liver Metastases. Other tumors that may manifest with liver-dominant metastases include soft tissue sarcomas such as gastrointestinal stromal tumor, breast carcinoma, and gynecologic malignancies. Treatment of these tumors may be undertaken in cases of limited progressive disease not responsive to systemic therapy. Preprocedure Considerations Risk Strati?cation. Scoring systems commonly used

2017 Society of Interventional Radiology

165. Ipilimumab (Yervoy) for unresectable malignant melanoma in paediatric patients

a type of white blood cells called lymphocytes or B-cells. It is the most common form of non-Hodgkin lymphoma among adults. DLBCL is an aggressive cancer and although it can be cured in more than 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 abnormally, they are unable to function and fight infection and reduce the production of healthy blood cells. The disease is chronic and develops slowly. Treatment for CLL is complex and depends on a number of factors, including extent of disease, previous treatment, patient’s age, symptoms and general state

2017 NIHR Innovation Observatory

166. 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

167. 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

168. 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

169. 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

170. 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

171. 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

172. 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

173. 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

174. 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

175. 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

176. 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

177. 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

178. 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

179. 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

180. Utility of FDG-PET imaging for risk stratification of pancreatic neuroendocrine tumours in MEN 1. Full Text available with Trip Pro

Utility of FDG-PET imaging for risk stratification of pancreatic neuroendocrine tumours in MEN 1. Patients with multiple endocrine neoplasia type 1 (MEN1) are at high risk of malignant pancreatic neuroendocrine tumors (pNETs). Structural imaging is typically used to screen for pNETs but is suboptimal for stratifying malignant potential.To determine the utility of fluorodeoxyglucose (18F) positron emission tomography/computed tomography (18F-FDG PET/CT) for predicting the malignant potential (...) FDG avidity [P < 0.001; sensitivity; 85.7% (95% confidence interval [CI], 48.7% to 99.3%); specificity, 95.2% (95% CI, 84.2% to 99.2%)].18F-FDG PET/CT is an effective screening modality in MEN1 for identifying pNETs of increased malignant potential. Surgical resection is recommended for FDG-avid pNETs.Copyright © 2017 Endocrine Society

2017 Journal of Clinical Endocrinology and Metabolism

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