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Multiple Endocrine Neoplasia Type 1

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221. Nivolumab and Ipilimumab in Treating Patients With Esophageal and Gastroesophageal Junction Adenocarcinoma Undergoing Surgery

table for MeSH terms Adenocarcinoma Esophageal Neoplasms Carcinoma Neoplasms, Glandular and Epithelial Neoplasms by Histologic Type Neoplasms Gastrointestinal Neoplasms Digestive System Neoplasms Neoplasms by Site Head and Neck Neoplasms Digestive System Diseases Esophageal Diseases Gastrointestinal Diseases Paclitaxel Albumin-Bound Paclitaxel Carboplatin Nivolumab Ipilimumab Antineoplastic Agents, Phytogenic Antineoplastic Agents Tubulin Modulators Antimitotic Agents Mitosis Modulators Molecular (...) Radiation: Radiation Therapy Undergo radiation Other Names: Cancer Radiotherapy Irradiate Irradiated irradiation Radiation Radiotherapeutics RADIOTHERAPY RT Therapy, Radiation Experimental: Arm B (carboplatin, paclitaxel, radiation therapy, nivolumab) Patients receive carboplatin, paclitaxel, and radiation therapy as in Arm A. Patients also receive nivolumab IV over 60 minutes on days 1 and 15. Cycles repeat every week for up to 5 weeks in the absence of disease progression or unacceptable toxicity

2018 Clinical Trials

222. Study to Evaluate the Safety and Tolerability of Avelumab in Combination With Other Anti-Cancer Therapies in Patients With Advanced Malignancies

Cavity and Pharynx Malignant Neoplasms of Male Genital Organs Malignant Neoplasms of Mesothelial and Soft Tissue Malignant Neoplasms of Respiratory and Intrathoracic Organs Malignant Neoplasms of Thyroid and Other Endocrine Glands Malignant Neoplasms of Urinary Tract Neoplasms of Uncertain or Unknown Behavior Drug: Avelumab Drug: Utomilumab Drug: PF-04518600 Radiation: Radiation Drug: Cisplatin Phase 1 Phase 2 Study Design Go to Layout table for study information Study Type : Interventional (Clinical (...) confirmed diagnosis of locally advanced cervical cancer confined to the pelvis only. Subjects must have measurable disease (RECIST v1.1) or patients may have bone metastatic disease evaluable by Prostate Cancer Working Group 2 (PCWG2) for subjects with metastatic castration-resistant prostate cancer (CRPC) or according to tumor evaluation criteria best suitable and accepted for the tumor type evaluated. Age >/= 18 years. Eastern Cooperative Oncology Group (ECOG) performance status of 0-1. Adequate

2017 Clinical Trials

223. Type 1 neurofibromatosis

is recommended. The disorder is progressive, although variable; it will worsen. Multiple organ systems are involved: skin, central nervous system, peripheral nerves, eyes, bones, gastrointestinal tract, and vascular and endocrine systems. Presentation in a young child may be subtle. Regular follow-up, especially of complex cases, is recommended to identify early lesions, especially malignant peripheral nerve sheath tumours. Definition Neurofibromatosis, type 1 (NF1) is an autosomal-dominant genetic disorder (...) Type 1 neurofibromatosis Type 1 neurofibromatosis - Symptoms, diagnosis and treatment | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Type 1 neurofibromatosis Last reviewed: February 2019 Last updated: November 2018 Summary Autosomal-dominant genetic disorder with the defining features of café au lait spots, multiple neurofibromas, and iris Lisch nodules. Diagnosis is made clinically; RNA-based NF1 mutation molecular testing to confirm the diagnosis

2017 BMJ Best Practice

224. Gaba Transcatheter Therapy for Hepatic Malignancy: Standardization of Terminology

sessions (ie, “scheduled” therapy) and the possibility of treating growing foci of local tumor progression months after the initial course of therapy (ie, “on-demand” therapy). A “window” for an initial treatment cycle for each catheter-based technique should be de?ned (eg, 1–3 mo)dependingon tumorsize, type, and number, as well as the rationale for therapy. The broad latitude given to this de?nition is purposeful, given the evolving consensus on de?ning more speci?c parameters because each disease (...) assessment provided the ?rst standardized method for quantifying morphologic response to Table 1. Response Outcome De?nitions for Radiologic Response Assessment Schemes Response Outcome WHO RECIST EASL mRECIST CR No measurable disease No measurable disease Disappearance of intratumoral arterial enhancement in all arterially enhancing tumors Disappearance of intratumoral arterial enhancementinalltarget tumors PR Z 50% decrease in sum of cross- products Z 30% decrease in sum of longest diameters Z 50

2016 Society of Interventional Radiology

225. Multiple Endocrine Neoplasia Type 1

(GENES, TUMOR SUPPRESSOR) on CHROMOSOME 11 (Locus: 11q13). Concepts Neoplastic Process ( T191 ) MSH ICD9 258.01 ICD10 SnomedCT 190566000 , 30664006 English MEA I , MEN I , Wermer Syndrome , MEA 1 , MEN 1 , MEN1 , Multiple Endocrine Neoplasia Type 1 , Multiple Endocrine Neoplasia Type I , Multiple Endocrine Neoplasms Type 1 , Neoplasia, Multiple Endocrine Type 1 , Neoplasms, Multiple Endocrine Type 1 , Neoplasms, Multiple Endocrine Type I , MEN syndrome type 1 , Multiple endoc neoplas type 1 , MEA (...) 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

2015 FP Notebook

226. The effectiveness of a structured transition program from a pediatric to an adult endocrine clinic for patients with type 1 diabetes mellitus

The effectiveness of a structured transition program from a pediatric to an adult endocrine clinic for patients with type 1 diabetes mellitus Print | PDF PROSPERO This information has been provided by the named contact for this review. CRD has accepted this information in good faith and registered the review in PROSPERO. CRD bears no responsibility or liability for the content of this registration record, any associated files or external websites. Email salutation (e.g. "Dr Smith" or "Joanne (...) and abstract, followed by full-text screening of the eligible articles for final inclusion. In each phase, 2 observers will independently assess each article. Discrepancies will be resolved through discussion, or by consulting a third investigator. ">Procedure for study selection Example : Title-abstract screening: 1. Not an original full research paper (e.g. review, editorial) 2. Not an in vivo animal study 3. No metastases/ only primary tumor 4. No control group 5. Combination therapy or contamination 6

2018 PROSPERO

227. Pharmacological Management of Osteoporosis in Postmenopausal Women: An Endocrine Society Guideline Update Full Text available with Trip Pro

bisphosphonates or denosumab are not appropriate or with a high risk of breast cancer. (1|⊕⊕⊕⊕) 6. Menopausal Hormone Therapy and Tibolone 6.1 In postmenopausal women at high risk of fracture and with the patient characteristics below, we suggest menopausal hormone therapy, using estrogen-only therapy in women with hysterectomy, to prevent all types of fractures. Patient characteristics: Under 60 years of age or < 10 years past menopause; at low risk of deep vein thrombosis; those in whom bisphosphonates (...) A.1 In postmenopausal women with osteoporosis at very high risk of fracture, such as those with severe osteoporosis ( ie, low T-score < −2.5 and fractures) or multiple vertebral fractures, we recommend romosozumab treatment for up to 1 year for the reduction of vertebral, hip, and nonvertebral fractures. Technical remark: The recommended dosage is 210 mg monthly by subcutaneous injection for 12 months. Women at high risk of cardiovascular disease and stroke should not be considered for romosozumab

2020 The Endocrine Society

228. European Society of Endocrinology Clinical Practice Guideline: Endocrine work-up in obesity

Symptoms and signs of hypogonadism LH FSH testosterone Androgen excess (women) Common Central obesity Irregular menses Hirsutism Acanthosis nigricans LH FSH oestradiol testosterone Cushing’s disease or Cushing’s syndrome Rare Central obesity Hypertension Type 2 diabetes 1 mg ODST Drug-induced endocrine dysfunction (e.g. lithium, anti-depressants, antipsychotics, glucocorticoids…) Common Psychiatric disorders Glucocorticoid therapy 1 mg ODST to exclude Cushing syndrome (except in glucocorticoid use (...) stimulation test Hypothalamic obesity associated with Genetic Syndromes Extremely rare Hypogonadism (hypogonadism or hypergonadotropic) or variable gonadal function. dysmorphic syndrome, mental and grow retardation Leptin (leptin resistance) (7); genetic testing Hypothalamic obesity acquired (hypothalamic lesions or, tumors) Rare Severe hyperphagia Possible multiple endocrine abnormalities Brain CT or MRI (Severe) hypothyroidism Rare Mixedematous features Concurrent autoimmune diseases FT4 TSH Insulinoma

2020 European Society of Endocrinology

229. Intravenous Doxorubicin Nanoparticles for Advanced Hepatocellular Carcinoma

as these outputs are produced as required for our stakeholders. > > > Intravenous Doxorubicin Nanoparticles for Advanced Hepatocellular Carcinoma Intravenous Doxorubicin Nanoparticles for Advanced Hepatocellular Carcinoma July 2017 Doxorubicin Nanoparticles are an injectable drug made up of microscopic polymer based spheres containing the drug doxorubicin. Doxorubicin, a chemotherapy drug used to treat many types of cancer, slows or stops the growth of cancer cells by blocking an enzyme called topoisomerase (...) 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

2017 NIHR Innovation Observatory

230. Pembrolizumab (Keytruda) for endometrial carcinoma – second line

are produced as required for our stakeholders. > > > Pembrolizumab (Keytruda) for endometrial carcinoma – second line Pembrolizumab (Keytruda) for endometrial carcinoma – second line March 2017 Pembrolizumab is a new drug to treat endometrial carcinoma, which is a type of cancer that begins in the womb. The most common symptoms of this cancer are post-menopausal or irregular vaginal bleeding. Pembrolizumab is given straight into the bloodstream, and if licensed for use in the UK, it could improve survival (...) 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

2017 NIHR Innovation Observatory

231. Pembrolizumab (Keytruda) for locally advanced or metastatic urothelial carcinoma in adults who are not eligible for cisplatin-containing chemotherapy – first line

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 of health. Patients whose CLL is not causing any symptoms or is getting worse only very

2017 NIHR Innovation Observatory

232. Landscape of CDKN1B Mutations in Luminal Breast Cancer and Other Hormone-Driven Human Tumors Full Text available with Trip Pro

Landscape of CDKN1B Mutations in Luminal Breast Cancer and Other Hormone-Driven Human Tumors The CDKN1B gene encodes for the p27Kip1 protein, firstly characterized as a cyclin dependent kinase (CDK)-inhibitor. Germline CDKN1B pathogenic variants have been described in hereditary tumors, such as multiple endocrine neoplasia (MEN)-like syndromes and familial prostate cancer. Despite its central role in tumor progression, for a long time it has been proposed that CDKN1B was very rarely somatically (...) intestine neuroendocrine tumors. However, a clear picture of the extent and significance of CDKN1B mutations in human malignances is still lacking. To fill this gap, we interrogated the COSMIC, ICGC, cBioPortal, and TRANSFAC data portals and current literature in PubMed, and reviewed the mutational spectrum of CDKN1B in human cancers, interpreting the possible impact of these mutations on p27Kip1 protein function and tumor onset and progression.

2018 Frontiers in endocrinology

233. Melatonin: An Anti-Tumor Agent in Hormone-Dependent Cancers Full Text available with Trip Pro

Melatonin: An Anti-Tumor Agent in Hormone-Dependent Cancers Melatonin (N-acetyl-5-methoxytryptamine) is a hormone synthesized and secreted by the pineal gland mainly during the night, since light exposure suppresses its production. Initially, an implication of this indoleamine in malignant disease was described in endocrine-responsive breast cancer. Data from several clinical trials and multiple experimental studies performed both in vivo and in vitro have documented that the pineal hormone (...) receptors MT-1 and MT-2, or else intracellular actions targeting molecules such as calmodulin, or binding intranuclear receptors. Similar results have been obtained in prostate (regulation of enzymes involved in androgen synthesis and modulation of androgen receptor levels and activity) and ovary cancer. Thus, tumor metabolism, gene expression, or epigenetic modifications are modulated, cell growth is impaired and angiogenesis and metastasis are inhibited. In the last decade, many more reports have

2018 International journal of endocrinology

234. GPCRomics: GPCR Expression in Cancer Cells and Tumors Identifies New, Potential Biomarkers and Therapeutic Targets Full Text available with Trip Pro

the expression of endoGPCRs (which respond to endogenous molecules such as hormones, neurotransmitters and metabolites) in multiple types of cancer cells. Using TaqMan qPCR arrays to quantify the mRNA expression of ∼340 such GPCRs, we found that human chronic lymphocytic leukemia (CLL) cells/stromal cells associated with CLL, breast cancer cell lines, colon cancer cell lines, pancreatic ductal adenocarcinoma (PDAC) cells, cancer associated fibroblasts (CAFs), and PDAC tumors express 50 to >100 GPCRs (...) GPCRomics: GPCR Expression in Cancer Cells and Tumors Identifies New, Potential Biomarkers and Therapeutic Targets G protein-coupled receptors (GPCRs), the largest family of targets for approved drugs, are rarely targeted for cancer treatment, except for certain endocrine and hormone-responsive tumors. Limited knowledge regarding GPCR expression in cancer cells likely has contributed to this lack of use of GPCR-targeted drugs as cancer therapeutics. We thus undertook GPCRomic studies to define

2018 Frontiers in pharmacology

235. Radiologic Management of Hepatic Malignancy

Radiologic Management of Hepatic Malignancy Date of origin: 2007 Last review date: 2015 ACR Appropriateness Criteria ® 1 Radiologic Management of Hepatic Malignancy American College of Radiology ACR Appropriateness Criteria ® Clinical Condition: Radiologic Management of Hepatic Malignancy Variant 1: Hepatocellular carcinoma: Solitary tumor 5 cm. Treatment/Procedure Rating Comments Systemic chemotherapy 6 Consider this procedure for patients not amenable to other localized therapies. Resection 5 (...) by interventional radiologists to treat hepatic malignancy. These treatments include direct tumor ablation via chemical or thermal means and endovascular techniques such as embolization, chemoembolization, and radioembolization with yttrium-90 (Y90). The role of these treatments in the management of primary and secondary hepatic malignancy is reviewed below. Discussion by Variant Variants 1–3: Primary hepatic malignancy; hepatocellular carcinoma Despite marked advances in interventional oncology over the past

2015 American College of Radiology

236. The role of endoscopy in the management of premalignant and malignant conditions of the stomach

-64 Gastric carcinoid tumors Gastric carcinoid tumors can be classi?ed as type 1 (multifocal, well differentiated, associated with type A chronicatrophicgastritis),type2(multifocal,welldifferenti- ated, associated with Zollinger-Ellison syndrome and multi- ple endocrine neoplasia type 1), type 3 (solitary, well differentiated, sporadic), and type 4 (solitary, poorly differ- entiated). Endoscopic evaluation should include a descrip- tion of carcinoid size, number, and anatomic distribution. Gastric (...) infection is identi?ed, eradication should be performed. It remains controversial whetherempiricHpyloritreatmentshouldbeadministered when GIM is diagnosed. Pernicious anemia The prevalence of gastric adenocarcinoma in patients with pernicious anemia, now considered to be associated with type A atrophic gastritis, 54 is reported to be 1% to 3%. 55 Most studies have shown a2- to3-fold increased inci- dence of gastric cancer in patients with pernicious ane- mia, 56-61 although a large U.S. population-based

2015 American Society for Gastrointestinal Endoscopy

237. RET Cys634Arg mutation confers a more aggressive multiple endocrine neoplasia type 2A phenotype than Cys634Tyr mutation. Full Text available with Trip Pro

RET Cys634Arg mutation confers a more aggressive multiple endocrine neoplasia type 2A phenotype than Cys634Tyr mutation. Specific germline mutations in the RET proto-oncogene are correlated with clinical features in multiple endocrine neoplasia type 2A (MEN2A); however, data are scarce regarding differences in clinical profiles dependent on the type of nucleotide and amino acid substitution at the same codon. We aimed to analyse differences in clinical risk profiles and outcomes among different (...) amino acids encoded by codon 634.The study was retrospective and multicentric.We collected data included in the Spanish Online National Database from patients with MEN2A carrying a RET proto-oncogene mutation on codon 634. The mean follow-up time was 7.6±6.9 years (1-32).Patients (n=173) from 49 unrelated families were C634Y carriers, and 26 patients from eight different families had C634R mutation. We found higher penetrance of medullary thyroid carcinoma, phaeochromocytoma and hyperparathyroidism

2014 European Journal of Endocrinology

238. Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study. (Abstract)

Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study. The prevention of medullary thyroid cancer in patients with multiple endocrine neoplasia type 2 syndrome has demonstrated the ability of molecular diagnosis and prophylactic surgery to improve patient outcomes. However, the other major neoplasia associated with multiple endocrine neoplasia type 2 (...) of surgery was patient driven. The primary aim of our analysis was to compare disease-free survival after either adrenal-sparing surgery or adrenalectomy.1210 patients with multiple endocrine neoplasia type 2 were included in our database, 563 of whom had phaeochromocytoma. Treatment was adrenalectomy in 438 (79%) of 552 operated patients, and adrenal-sparing surgery in 114 (21%). Phaeochromocytoma recurrence occurred in four (3%) of 153 of the operated glands after adrenal-sparing surgery after 6-13

2014 Lancet Oncology

239. The association between Hirschsprung's disease and multiple endocrine neoplasia type 2a: a systematic review. (Abstract)

The association between Hirschsprung's disease and multiple endocrine neoplasia type 2a: a systematic review. The co-occurrence of Hirschsprung's disease (HSCR) and multiple endocrine neoplasia type 2 (MEN2) is a relatively rare event. The basis for this association is the presence of a "Janus" mutation in the RET proto-oncogene--a mutation that acts simultaneously as both a gain-in-function and a loss-of-function mutation. To date, four mutations in the exon 10 region of RET that are known

2014 Pediatric surgery international

240. Primary hyperparathyroidism in multiple endocrine neoplasia type 1: when to perform surgery? Full Text available with Trip Pro

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

2012 Clinics

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