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Multiple Endocrine Neoplasia Type 1

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281. TAK228 With Carbo and Taxol in Advanced Malignancies

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 Malignant Neoplasms Stated as Primary Lymphoid Haematopoietic Drug: TAK-228 Drug: Paclitaxel Drug: Carboplatin Phase 1 Study Design Go to Layout table for study information Study Type : Interventional (Clinical Trial) Estimated Enrollment : 50 participants Intervention Model: Single Group Assignment Masking: None (Open Label (...) hepatitis C infection. Any serious medical or psychiatric illness that could, in the investigator's opinion, potentially interfere with the completion of treatment according to this protocol. Diagnosed or treated for another malignancy within 2 years before administration of the first dose of study drug, or previously diagnosed with another malignancy and have any evidence of residual disease. Patients with non-melanoma skin cancer or carcinoma in situ of any type are not excluded if they have undergone

2018 Clinical Trials

282. Specialized Immune Cells (nCTLs) and a Vaccine (Alpha-type-1 Polarized Dendritic Cells) in Treating Patients With Stage II-IV Ovarian, Fallopian Tube, or Primary Peritoneal Cancer

and eliminate tumor cells. These "educated" immune cells are then given back to the patient. An alpha-type-1 polarized dendritic cell vaccine is another population of "educated" immune cells that work to support the infused nCTLs. Giving nCTLS with a dendritic cell vaccine may work better in treating patients with ovarian, fallopian tube, or primary peritoneal cancer. Condition or disease Intervention/treatment Phase Stage II Fallopian Tube Cancer AJCC v8 Stage II Ovarian Cancer AJCC v8 Stage II Primary (...) relevant MeSH terms: Layout table for MeSH terms Ovarian Neoplasms Carcinoma, Ovarian Epithelial Fallopian Tube Neoplasms Peritoneal Neoplasms Endocrine Gland Neoplasms Neoplasms by Site Neoplasms Ovarian Diseases Adnexal Diseases Genital Diseases, Female Genital Neoplasms, Female Urogenital Neoplasms Endocrine System Diseases Gonadal Disorders Carcinoma Neoplasms, Glandular and Epithelial Neoplasms by Histologic Type Fallopian Tube Diseases Abdominal Neoplasms Digestive System Neoplasms Digestive

2018 Clinical Trials

283. The Characterization of Pheochromocytoma and Its Impact on Overall Survival in Multiple Endocrine Neoplasia Type 2. Full Text available with Trip Pro

The Characterization of Pheochromocytoma and Its Impact on Overall Survival in Multiple Endocrine Neoplasia Type 2. Pheochromocytoma (PHEO) occurs in 50% of patients with multiple endocrine neoplasia type 2 (MEN2). It is unknown if the presence of PHEO is associated with more aggressive medullary thyroid cancer (MTC).To present our experience with MEN2 PHEO and evaluate whether PHEO impacts MTC overall survival in patients with RET codon 634 mutations.We performed a retrospective chart review (...) of MEN2 patients at MD Anderson Cancer Center from 1960 through 2012.The study group comprised 85 patients (group 1) with MEN2-associated PHEO. Of these, 59 patients (subgroup 1) with RET codon 634 mutations were compared to 48 patients (group 2) with RET codon 634 mutations, but without MEN2-associated PHEO.Of 85 patients with MEN2 and PHEO, 70 had MEN2A and 15 had MEN2B. Median age at PHEO diagnosis was 32 years. The initial manifestation of MEN2 was MTC in 60% of patients, synchronous MTC and PHEO

2013 Journal of Clinical Endocrinology and Metabolism

284. Pheochromocytoma in an 8-year-old patient with multiple endocrine neoplasia type 2A: Implications for screening. Full Text available with Trip Pro

Pheochromocytoma in an 8-year-old patient with multiple endocrine neoplasia type 2A: Implications for screening. Childhood pheochromocytoma in the setting of multiple endocrine neoplasia type 2 (MEN2) remains rare and has not been reported under the age of 12. We present an 8-year-old female with known MEN 2A, C634Y RET mutation, diagnosed with a 6 cm pheochromocytoma requiring laparoscopic adrenalectomy. Given this patient's age at diagnosis, screening guidelines should recommend annual

2013 Journal of Surgical Oncology

285. Multiple endocrine neoplasia type 2A due to an exon 8 (G533C) mutation in a large North American kindred. (Abstract)

Multiple endocrine neoplasia type 2A due to an exon 8 (G533C) mutation in a large North American kindred. Medullary thyroid cancer, although most commonly sporadic, may be part of the multiple endocrine neoplasia type 2 (MEN2) syndromes, generally due to mutations in the RET proto-oncogene. The majority of these mutations are located in exons 10, 11, and 13-16. More rarely, mutations in other exons have been described. We report for the first time a family from the United States with a rare (...) mutation involving exon 8 of the RET proto-oncogene, corresponding to a p.Gly533Cys substitution (G533C) leading to the development of MEN2A syndrome in several affected family members. This mutation had only been previously described in a large family in Brazil and in 7.75% of patients with apparently sporadic medullary thyroid cancer (MTC) in Greece.Given a strong index of suspicion, a genetic analysis to evaluate for uncommon mutations in the RET proto-oncogene identified the presence of the G533C

2013 Thyroid

286. Sotagliflozin tablets as an adjuct therapy to insulin for Type 1 diabetes mellitus

removes excess cholesterol, reducing the risk of cardiovascular disease. Apabetalone has the potential to affect multiple processes which contribute to cardiovascular disease, with the overall result of reducing the risk of a major adverse cardiac event in diabetic patients. If licensed, apabetalone may offer an additional preventive treatment option for major adverse cardiac events in patients with type 2 diabetes mellitus, high-risk a high risk of cardiovascular disease and low HDL. August 2018 (...) be left unchanged. This iframe contains the logic required to handle AJAX powered Gravity Forms. Get Alerts Subscribe to new content email alerts Email * Interested groups * Anaesthetics Pain Relief and ITU Cancer and Palliative Care Cardiovascular Disease and Vascular Surgery Dental and Oral Health and Maxillo-facial Surgery Ear Nose and Throat Emergency Care Endocrine Nutritional and Metabolic Eye Disease Gastrointestinal Pancreatic and Liver Disease Gynaecology Womens Sexual Health and Benign

2017 NIHR Innovation Observatory

287. Triheptanoin (UX-007) for glucose transporter type 1 deficiency syndrome (de vivo disease) – first line

is in clinical development for children with neurofibromatosis type 1 (NF1), also called von Recklinghausen’s disease. NF1 is a rare genetic disorder characterized by the development of multiple benign tumours of nerves and skin and areas of abnormal skin colour. NF1 is caused by mutation in a gene that regulates the production of a … November 2018 Edaravone as an intravenous injection is in clinical development for people with amyotrophic lateral sclerosis (ALS). ALS is a neurological condition that affects (...) Triheptanoin (UX-007) for glucose transporter type 1 deficiency syndrome (de vivo disease) – first line Triheptanoin (UX-007) for glucose transporter type 1 deficiency syndrome (de vivo disease) – first line | Innovation Observatory toggle menu Menu Search View All Filter by Speciality Filter by Year Filter by Category This search function provides links to outputs produced by NIHR Innovation Observatory. These are briefing notes or reports on new or repurposed technologies. This search

2017 NIHR Innovation Observatory

288. Enhancer-mediated oncogenic function of the menin tumor suppressor in breast cancer Full Text available with Trip Pro

Enhancer-mediated oncogenic function of the menin tumor suppressor in breast cancer While the multiple endocrine neoplasia type 1 (MEN1) gene functions as a tumor suppressor in a variety of cancer types, we explored its oncogenic role in breast tumorigenesis. The MEN1 gene product menin is involved in H3K4 trimethylation and co-activates transcription. We integrated ChIP-seq and RNA-seq data to identify menin target genes. Our analysis revealed that menin-dependent target gene promoters display (...) looping to distal enhancers that are bound by menin, FOXA1 and GATA3. In this fashion, MEN1 co-regulates a proliferative breast cancer-specific gene expression program in ER+ cells. In primary mammary cells, MEN1 exerts an anti-proliferative function by regulating a distinct expression signature. Our findings clarify the cell-type-specific functions of MEN1 and inform the development of menin-directed treatments for breast cancer.Copyright © 2017 The Author(s). Published by Elsevier Inc. All rights

2017 Cell reports

289. Bisphenol A activates EGFR and ERK promoting proliferation, tumor spheroid formation and resistance to EGFR pathway inhibition in estrogen receptor-negative inflammatory breast cancer cells Full Text available with Trip Pro

Bisphenol A activates EGFR and ERK promoting proliferation, tumor spheroid formation and resistance to EGFR pathway inhibition in estrogen receptor-negative inflammatory breast cancer cells Emerging evidence from epidemiological studies suggests a link between environmental chemical exposure and progression of aggressive breast cancer subtypes. Of all clinically distinct types of breast cancers, the most lethal phenotypic variant is inflammatory breast cancer (IBC). Overexpression of epidermal (...) to identify chemicals that can potentiate EGFR mitogenic effects in IBC. Herein, we assessed in ER-negative IBC cells a subset of chemicals from the EPA ToxCast set for their effect on EGFR activation and in multiple cancer phenotypic assays. We demonstrated that endocrine-disrupting chemicals such as bisphenol A (BPA) and 2,2-bis(p-hydroxyphenyl)-1,1,1-trichloroethane can increase EGFR/ERK signaling. BPA also caused a corresponding increase in expression of SOD1 and anti-apoptotic Bcl-2, key markers

2017 Carcinogenesis

290. Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®): Health Professional Version

Cancer 64 (8): , 2017. [ ] Warren KE, Vezina G, Poussaint TY, et al.: Response assessment in medulloblastoma and leptomeningeal seeding tumors: recommendations from the Response Assessment in Pediatric Neuro-Oncology committee. Neuro Oncol 20 (1): 13-23, 2018. [ ] [ ] Fouladi M, Gajjar A, Boyett JM, et al.: Comparison of CSF cytology and spinal magnetic resonance imaging in the detection of leptomeningeal disease in pediatric medulloblastoma or primitive neuroectodermal tumor. J Clin Oncol 17 (10 (...) of intermediate differentiation, has been identified but is not considered an embryonal tumor and primarily arises in adults.[ ] The PDQ childhood brain tumor treatment summaries are organized primarily according to the World Health Organization (WHO) classification of nervous system tumors.[ ] For a full description of the classification of nervous system tumors and a link to the corresponding treatment summary for each type of brain tumor, refer to the PDQ summary on . Anatomy Figure 1. Anatomy

2018 PDQ - NCI's Comprehensive Cancer Database

291. Childhood Vascular Tumors Treatment (PDQ®): Health Professional Version

and ) but the ISSVA classification uses more precise terminology and phenotypes that have been agreed upon by the members of ISSVA. Table 1. 2013 World Health Organization Classification of Vascular Tumors Category Vascular Tumor Type Benign Hemangioma Epithelioid hemangioma Angiomatosis Lymphangioma Intermediate (locally aggressive) Kaposiform hemangioendothelioma Intermediate (rarely metastasizing) Retiform hemangioendothelioma Papillary intralymphatic angioendothelioma Composite hemangioendothelioma Kaposi (...) sarcoma Malignant Epithelioid hemangioendothelioma Angiosarcoma of soft tissue a Adapted from Fletcher et al.[ ] Table 2. 2014 International Society for the Study of Vascular Anomalies (ISSVA) Classification of Vascular Tumors a Category Vascular Tumor Type Benign Infantile hemangioma/hemangioma of infancy Congenital hemangioma —Rapidly involuting (RICH) —Non-involuting (NICH) —Involuting (PICH) Tufted angioma Spindle cell hemangioma Pyogenic granuloma (also known as lobular capillary hemangioma

2018 PDQ - NCI's Comprehensive Cancer Database

292. Neoplasms of the Endocrine Pancreas (Follow-up)

analysis of nine endocrine neoplasms of the pancreas. Cancer Genet Cytogenet . 1994 Oct. 77(1):55-9. . Zeiger MA, Norton JA. Gs alpha--identification of a gene highly expressed by insulinoma and other endocrine tumors. Surgery . 1993 Aug. 114(2):458-62; discussion 462-3. . Eubanks PJ, Sawicki MP, Samara GJ, et al. Pancreatic endocrine tumors with loss of heterozygosity at the multiple endocrine neoplasia type I locus. Am J Surg . 1997 Jun. 173(6):518-20. . Miller JA, Norton JA. Multiple endocrine (...) , the rate of tumor growth is still a major determinant of survival in patients with these lesions. [ ] Because these tumors usually grow slowly and have a relatively low metastatic potential and because no specific criteria have been defined to predict their behavior, the distinction between benign and malignant neoplasms is based on the presence of metastatic disease; therefore, long-term clinical follow-up is often required. [ , ] Neoplasms of the endocrine pancreas have patterns of initial tumor

2014 eMedicine.com

293. Neoplasms of the Endocrine Pancreas (Overview)

. 1985: 85. Long PP, Hruban RH, Lo R, et al. Chromosome analysis of nine endocrine neoplasms of the pancreas. Cancer Genet Cytogenet . 1994 Oct. 77(1):55-9. . Zeiger MA, Norton JA. Gs alpha--identification of a gene highly expressed by insulinoma and other endocrine tumors. Surgery . 1993 Aug. 114(2):458-62; discussion 462-3. . Eubanks PJ, Sawicki MP, Samara GJ, et al. Pancreatic endocrine tumors with loss of heterozygosity at the multiple endocrine neoplasia type I locus. Am J Surg . 1997 Jun. 173(6 (...) are characterized as the sporadic form. The second form affects kindreds with the multiple endocrine neoplasia type 1 (MEN 1) syndrome in a pattern of autosomal dominant inheritance. [ ] Approximately 80% of individuals with MEN 1 syndrome have one or more pancreatic neoplasms in their lifetime; gastrinoma and insulinoma are the most commonly identified lesions. [ ] Clinically recognized neoplasms of the endocrine pancreas are rare, with an overall annual incidence in the United States of 3-10 cases per million

2014 eMedicine.com

294. Neoplasms of the Endocrine Pancreas (Treatment)

, Sawicki MP, Samara GJ, et al. Pancreatic endocrine tumors with loss of heterozygosity at the multiple endocrine neoplasia type I locus. Am J Surg . 1997 Jun. 173(6):518-20. . Miller JA, Norton JA. Multiple endocrine neoplasia. Cancer Treat Res . 1997. 90:213-25. . Nakeeb A, Lillemoe KD, Yeo CJ. Neoplasms of the exocrine pancreas. In: Greenfield LJ, Mulholland MW, Oldham KT, eds. Surgery: Scientific Principles and Practice. 3rd ed. Philadelphia, Pa: . Lippincott-Raven. 2001:885-99. [Guideline] NCCN (...) and its mesentery; peripancreatic lymph nodes; and, in women, the reproductive tract. [ ] The goals of surgical therapy for pancreatic endocrine neoplasms include (1) controlling the symptoms of hormone excess, (2) safely resecting the maximal amount of tumor mass possible, and (3) preserving the maximal amount of pancreatic parenchyma possible. [ ] Surgical management of the primary tumor is similar for the different types of pancreatic endocrine neoplasms. Surgical treatments may include

2014 eMedicine.com

295. Neoplasms of the Endocrine Pancreas (Diagnosis)

. 1985: 85. Long PP, Hruban RH, Lo R, et al. Chromosome analysis of nine endocrine neoplasms of the pancreas. Cancer Genet Cytogenet . 1994 Oct. 77(1):55-9. . Zeiger MA, Norton JA. Gs alpha--identification of a gene highly expressed by insulinoma and other endocrine tumors. Surgery . 1993 Aug. 114(2):458-62; discussion 462-3. . Eubanks PJ, Sawicki MP, Samara GJ, et al. Pancreatic endocrine tumors with loss of heterozygosity at the multiple endocrine neoplasia type I locus. Am J Surg . 1997 Jun. 173(6 (...) are characterized as the sporadic form. The second form affects kindreds with the multiple endocrine neoplasia type 1 (MEN 1) syndrome in a pattern of autosomal dominant inheritance. [ ] Approximately 80% of individuals with MEN 1 syndrome have one or more pancreatic neoplasms in their lifetime; gastrinoma and insulinoma are the most commonly identified lesions. [ ] Clinically recognized neoplasms of the endocrine pancreas are rare, with an overall annual incidence in the United States of 3-10 cases per million

2014 eMedicine.com

296. Surgical Curability of Medullary Thyroid Cancer in Multiple Endocrine Neoplasia 2B: A Changing Perspective. (Abstract)

Surgical Curability of Medullary Thyroid Cancer in Multiple Endocrine Neoplasia 2B: A Changing Perspective. This investigation aimed at exploring the suitability of nonendocrine manifestations preceding medullary thyroid cancer (MTC) for early diagnosis of multiple endocrine neoplasia type 2B (MEN 2B).MEN 2B patients, running a high risk of metastatic MTC, must be diagnosed early for biochemical cure.Forty-four MEN 2B patients carrying inherited (3 patients) and de novo (41 patients) M918T RET (...) mutations were examined for signs and symptoms prompting MEN 2B.All 3 patients with inherited mutations were diagnosed before the age of 1 year and cured of their C-cell disease. Among 41 patients with de novo mutations, MEN 2B was diagnosed in 12 patients after recognition of nonendocrine manifestations [intestinal ganglioneuromatosis (6 patients), oral symptoms (5 patients), ocular ("tearless crying") (4 patients), and skeletal stigmata (1 patient) alone or concomitantly]. In the remaining 29 patients

2013 Annals of Surgery

297. Merkel Cell Carcinoma Treatment (PDQ®): Health Professional Version

carcinoma and non-MCC tumors. J Cutan Pathol 37 (1): 28-34, 2010. [ ] Kassem A, Technau K, Kurz AK, et al.: Merkel cell polyomavirus sequences are frequently detected in nonmelanoma skin cancer of immunosuppressed patients. Int J Cancer 125 (2): 356-61, 2009. [ ] Foulongne V, Dereure O, Kluger N, et al.: Merkel cell polyomavirus DNA detection in lesional and nonlesional skin from patients with Merkel cell carcinoma or other skin diseases. Br J Dermatol 162 (1): 59-63, 2010. [ ] DeCaprio JA: Does (...) . Cancer Epidemiol Biomarkers Prev 8 (2): 153-8, 1999. [ ] Lemos B, Nghiem P: Merkel cell carcinoma: more deaths but still no pathway to blame. J Invest Dermatol 127 (9): 2100-3, 2007. [ ] Albores-Saavedra J, Batich K, Chable-Montero F, et al.: Merkel cell carcinoma demographics, morphology, and survival based on 3870 cases: a population based study. J Cutan Pathol 37 (1): 20-7, 2010. [ ] Heath M, Jaimes N, Lemos B, et al.: Clinical characteristics of Merkel cell carcinoma at diagnosis in 195 patients

2018 PDQ - NCI's Comprehensive Cancer Database

298. The PI3K/Akt Pathway in Tumors of Endocrine Tissues Full Text available with Trip Pro

The PI3K/Akt Pathway in Tumors of Endocrine Tissues The phosphatidylinositol 3-kinase (PI3K)/Akt pathway is a key driver in carcinogenesis. Defects in this pathway in human cancer syndromes such as Cowden's disease and Multiple Endocrine Neoplasia result in tumors of endocrine tissues, highlighting its importance in these cancer types. This review explores the growing evidence from multiple animal and in vitro models and from analysis of human tumors for the involvement of this pathway (...) in the following: thyroid carcinoma subtypes, parathyroid carcinoma, pituitary tumors, adrenocortical carcinoma, phaeochromocytoma, neuroblastoma, and gastroenteropancreatic neuroendocrine tumors. While data are not always consistent, immunohistochemistry performed on human tumor tissue has been used alongside other techniques to demonstrate Akt overactivation. We review active Akt as a potential prognostic marker and the PI3K pathway as a therapeutic target in endocrine neoplasia.

2016 Frontiers in endocrinology

299. HEREDITARY SYNDROMES PREDISPOSING TO ENDOCRINE TUMORS AND THEIR SKIN MANIFESTATIONS Full Text available with Trip Pro

HEREDITARY SYNDROMES PREDISPOSING TO ENDOCRINE TUMORS AND THEIR SKIN MANIFESTATIONS We often think of the lentiginoses, phacomatoses and other neurocutaneous syndromes as conditions that affect the skin and also predispose to a variety of tumors. However, we rarely think of Peutz-Jeghers syndrome (PJS), Carney complex (CNC), Cowden disease (CD), neurofibromatosis type-1 (NF-1) or tuberous sclerosis (TSC) as conditions that are multiple endocrine neoplasias (MEN). Indeed, all of these conditions (...) predispose to a variety of endocrine tumors, in addition to many other neoplasms. On the other hand, the classic MENs, type 1 and 2 (MEN-1 and MEN-2, respectively) are almost never thought in terms of their skin manifestations. In this review, we present extensively the MEN-1, MEN-2 and PJS syndromes, and briefly refer to CD, NF-1, and TSC. CNC is discussed in another article in this journal issue.

2016 Reviews in endocrine & metabolic disorders

300. Multiple Endocrine Neoplasia Type 2

that encodes a RECEPTOR PROTEIN-TYROSINE KINASE. It is an autosomal dominant inherited disease. Concepts Neoplastic Process ( T191 ) MSH ICD9 258.02 ICD10 SnomedCT 61808009 English MEA II , MEN II , Sipple Syndrome , MEA 2a , MEA IIa , MEN 2 , MEN 2a , MEN IIa , MEN2a , Multiple Endocrine Neoplasia Type 2a , Multiple Endocrine Neoplasms Type 2a , Neoplasia, Multiple Endocrine Type 2a , Neoplasms, Multiple Endocrine Type 2a , MEN syndrome type 2A , Multiple endoc neoplas type 2A , MEA Type 2a , MEN Type 2a (...) Multiple Endocrine Neoplasia Type 2 Multiple Endocrine Neoplasia Type 2 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

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