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Multiple Endocrine Neoplasia Type 1

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241. Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy Full Text available with Trip Pro

Surgical approach in patients with hyperparathyroidism in multiple endocrine neoplasia type 1: total versus partial parathyroidectomy Usually, primary hyperparathyroidism is the first endocrinopathy to be diagnosed in patients with multiple endocrine neoplasia type 1, and is also the most common one. The timing of the surgery and strategy in multiple endocrine neoplasia type 1/hyperparathyroidism are still under debate. The aims of surgery are to: 1) correct hypercalcemia, thus preventing (...) observed in multiple endocrine neoplasia type 1 than in sporadic hyperparathyroidism. The recurrence rate is strongly influenced by: 1) the lack of a pre-operative multiple endocrine neoplasia type 1 diagnosis; 2) the surgeon's experience; 3) the timing of surgery; 4) the possibility of performing intra-operative confirmation (histologic examination, rapid parathyroid hormone assay) of the curative potential of the surgical procedure; and, 5) the surgical strategy. Persistent hyperparathyroidism seems

2012 Clinics

242. Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center Full Text available with Trip Pro

multiglandular disease and it is surgically approached by either subtotal parathyroidectomy or total parathyroidectomy followed by parathyroid auto-implant to the forearm. In skilful hands, the efficacy of both approaches is similar and both should be complemented by prophylactic thymectomy. In a single academic center, 83 cases of hyperparathyroidism/ multiple endocrine neoplasia type 1 were operated on from 1987 to 2010 and our first surgical choice was total parathyroidectomy followed by parathyroid auto (...) Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center Most cases of sporadic primary hyperparathyroidism present disturbances in a single parathyroid gland and the surgery of choice is adenomectomy. Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical

2012 Clinics

243. Biochemical, bone and renal patterns in hyperparathyroidism associated with multiple endocrine neoplasia type 1. Full Text available with Trip Pro

in cases of hyperparathyroidism/multiple endocrine neoplasia type 1. Cortical bone mineral density in hyperparathyroidism/multiple endocrine neoplasia type 1 cases has only recently been examined, and early, severe and frequent bone mineral losses have been documented at this site. Early bone mineral losses are highly prevalent in the trabecular bone of patients with hyperparathyroidism/multiple endocrine neoplasia type 1. In summary, bone mineral disease in multiple endocrine neoplasia type 1 related (...) and mortality. In this article we review the few available studies on bone mineral and renal disturbances in the setting of hyperparathyroidism/multiple endocrine neoplasia type 1. We performed a meta-analysis of the available data on bone mineral and renal disease in cases of multiple endocrine neoplasia type 1-related hyperparathyroidism.

2012 Clinics (São Paulo, Brazil)

244. Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? Full Text available with Trip Pro

Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association? Diagnosis of multiple endocrine neoplasia type 1 (MEN1) is commonly based on clinical criteria, and confirmed by genetic testing. In patients without known MEN1-related germline mutations, the possibility of a casual association between two or more endocrine tumors cannot be excluded and subsequent management may be difficult to plan. We describe a very uncommon (...) pHPT were diagnosed and, since family history was negative, sporadic MEN1 was suspected. However, genetic testing revealed neither MEN-1 nor other gene mutations responsible for rarer cases of MEN1 (CDKN1B/p27 and other cyclin-dependent kinase inhibitor genes CDKN1A/p15, CDKN2C/p18, CDKN2B/p21). The patient underwent distal splenopancreatectomy and at the 4-month follow-up she showed complete remission of symptoms. Six months later, a thyroid nodule, suspected to be a malignant neoplasia, and two

2012 BMC Cancer

245. Partial Pancreaticoduodenectomy Can Provide Cure for Duodenal Gastrinoma Associated With Multiple Endocrine Neoplasia Type 1. (Abstract)

Partial Pancreaticoduodenectomy Can Provide Cure for Duodenal Gastrinoma Associated With Multiple Endocrine Neoplasia Type 1. To evaluate the outcome of pancreaticoduodenectomy (PD) versus non-PD resections for the treatment of gastrinoma in multiple endocrine neoplasia type 1.Gastrinoma in MEN1 is considered a rarely curable disease and its management is highly controversial both for timing and extent of surgery.Clinical characteristics, complications and outcomes of 27 prospectively collected (...) MEN1 patients with biochemically proven gastrinoma, who underwent surgery, were analyzed with special regard to the gastrinoma type and the initial operative procedure.Twenty-two (81%) patients with gastrinoma in MEN1 had duodenal gastrinomas and 5 patients (19%) had pancreatic gastrinomas. At the time of diagnosis, 21 (77%) gastrinomas were malignant (18 duodenal, 3 pancreatic), but distant metastases were only present in 4 (15%) patients. Patients with pancreatic gastrinomas underwent either

2012 Annals of Surgery

246. Preoperative Assessment of the Pancreas in Multiple Endocrine Neoplasia Type 1. (Abstract)

Preoperative Assessment of the Pancreas in Multiple Endocrine Neoplasia Type 1. Many serologic and radiographic modalities are used for monitoring multiple endocrine neoplasia type 1 (MEN 1) patients for pancreaticoduodenal neuroendocrine tumors (PNETs). We compared serum markers and imaging studies obtained preoperatively with the gross pathology and immunohistochemical findings and correlated preoperative testing with postoperative outcome.From 2000 to 2008, 52 MEN 1 patients [32 (62%) female (...) ). Overall survival was 89% at 5-year follow-up.Our study substantiates EUS as providing the highest preoperative sensitivity and PPV in assessing the presence of PNETs in MEN 1 patients. CT and octreotide scintigraphy can yield both false-positive and false-negative results. HPP, gastrin, and glucagon were the most commonly measured tumor markers in our series but did not always correlate with immunostaining. With an aggressive surgical approach, satisfactory rates of biochemical improvement and long

2012 World Journal of Surgery

247. Clinical Practice Guidelines for Multiple Endocrine Neoplasia Type 1 (MEN1). Full Text available with Trip Pro

Clinical Practice Guidelines for Multiple Endocrine Neoplasia Type 1 (MEN1). The aim was to provide guidelines for evaluation, treatment, and genetic testing for multiple endocrine neoplasia type 1 (MEN1).The group, which comprised 10 experts, including physicians, surgeons, and geneticists from international centers, received no corporate funding or remuneration.Guidelines were developed by reviews of peer-reviewed publications; a draft was prepared, reviewed, and rigorously revised at several (...) stages; and agreed-upon revisions were incorporated.MEN1 is an autosomal dominant disorder that is due to mutations in the tumor suppressor gene MEN1, which encodes a 610-amino acid protein, menin. Thus, the finding of MEN1 in a patient has important implications for family members because first-degree relatives have a 50% risk of developing the disease and can often be identified by MEN1 mutational analysis. MEN1 is characterized by the occurrence of parathyroid, pancreatic islet, and anterior

2012 Journal of Clinical Endocrinology and Metabolism

248. Post-surgical follow-up of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1 Full Text available with Trip Pro

Post-surgical follow-up of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1 The bone mineral density increments in patients with sporadic primary hyperparathyroidism after parathyroidectomy have been studied by several investigators, but few have investigated this topic in primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Further, as far as we know, only two studies have consistently evaluated bone mineral density values after (...) parathyroidectomy in cases of primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Here we revised the impact of parathyroidectomy (particularly total parathyroidectomy followed by autologous parathyroid implant into the forearm) on bone mineral density values in patients with primary hyperparathyroidism associated with multiple endocrine neoplasia type 1. Significant increases in bone mineral density in the lumbar spine and femoral neck values were found, although no short-term (15

2012 Clinics

249. Care of Adults with Neurofibromatosis Type 1

, unexplained neurologic symptoms (MPNST, brain tumors), and diaphoresis/palpitations (pheochromo- cytoma) are important. Although many issues in adults with NF1 can bemanagedbyaninternistorfamilyphysician,westronglyencourage evaluation by, and care coordination with, a specialized NF1 clinic. Genet Med advance online publication 26 April 2018 Key Words: breast cancer; cutaneous neurofibromas; malignant peripheral nerve sheath tumor; neurofibromatosis type 1; osteope- nia/osteoporosis Neurofibromatosis type (...) as a screen for MPNST, the full spectrum of NF1-associated malignancy, the natural history and treatment of NF1-associated osteoporosis and osteopenia, treatment of chronic pain, NF1-associated Parkinson’s disease and multiple sclerosis, and effective biomarkers and imaging strategies for tumor screening (e.g., pheochromocytoma, glioblastoma multiforme). Table 1 Assessment of adults with NF1 In addition to recommended age-and gender-specific screening and vaccinations, an annual general medical evaluation

2018 American College of Medical Genetics and Genomics

250. Metronomic Oral Vinorelbine Plus Anti-PD-L1/Anti-CTLA4 ImmunothErapy in Patients With Advanced Solid Tumours

of metronomic oral vinorelbine associated with durvalumab + tremelimumab combination immunotherapy for the treatment of advanced solid tumours. Condition or disease Intervention/treatment Phase Advanced Solid Tumours Breast Cancer Head and Neck Cancer Cervix Cancer Prostate Cancer Drug: Durvalumab + Tremelimumab + metronomic Vinorelbine Phase 1 Phase 2 Detailed Description: Methodology: The study divided in two parts: Phase I part: dose escalation study of metronomic oral vinorelbine associated (...) to comply with a baseline tumour biopsy (unless an archived biopsy of a secondary or a primary site of the current disease-collected within 3 months prior enrolment is available for research ; bone metastasis are accepted only when predominant extra-bone tissue is available), and with a series of blood samples throughout the study. Patient must be affiliated to a social health insurance. Exclusion Criteria: Other concurrent malignancies, except adequately treated cone-biopsied in situ carcinoma

2018 Clinical Trials

251. Genetics of Endocrine and Neuroendocrine Neoplasias (PDQ®): Health Professional Version

and jaw tumor syndrome associated with renal hamartomas and cystic kidney disease: linkage to 1q21-q32 and loss of the wild type allele in renal hamartomas. J Clin Endocrinol Metab 81 (12): 4204-11, 1996. [ ] Carpten JD, Robbins CM, Villablanca A, et al.: HRPT2, encoding parafibromin, is mutated in hyperparathyroidism-jaw tumor syndrome. Nat Genet 32 (4): 676-80, 2002. [ ] Marx SJ: Multiple endocrine neoplasia type 1. In: Vogelstein B, Kinzler KW, eds.: The Genetic Basis of Human Cancer. New York, NY (...) % [ ] Diabetes mellitus Diarrhea/steatorrhea Gallbladder disease Hypochlorhydria Weight loss MEN1 = multiple endocrine neoplasia type 1. Gastrinomas represent 50% of the gastrointestinal NETs in MEN1 and are the major cause of morbidity and mortality in MEN1 patients.[ , ] Gastrinomas are usually multicentric, with small (<0.5 cm) foci throughout the duodenum.[ ] Most result in peptic ulcer disease (Zollinger-Ellison syndrome), and half are malignant at the time of diagnosis.[ , , ] Nonfunctioning

2016 PDQ - NCI's Comprehensive Cancer Database

252. Oncogenetic testing for persons with hereditary endocrine cancer syndromes

assigned by CRD MeSH Adrenal Gland Neoplasms; Biomarkers, Tumor; Carcinoma; Endocrine Gland Neoplasms; Genetic Predisposition to Disease; Genetic Testing; Humans; Multiple Endocrine Neoplasia; Parathyroid Neoplasms; Pituitary Neoplasms; Thyroid Neoplasms Language Published English Country of organisation Belgium English summary An English language summary is available. Address for correspondence Belgian Health Care Knowledge Centre (KCE), Administrative Centre Botanique, Doorbuilding (10th floor (...) Oncogenetic testing for persons with hereditary endocrine cancer syndromes Oncogenetic testing for persons with hereditary endocrine cancer syndromes Oncogenetic testing for persons with hereditary endocrine cancer syndromes Vlayen J, Bex M, Bravenboer B, Claes K, Lapauw B, Persu A, Poppe K, Ullman U, Van Maerken T, Vroonen L, Poppe B Record Status This is a bibliographic record of a published health technology assessment from a member of INAHTA. No evaluation of the quality of this assessment

2015 Health Technology Assessment (HTA) Database.

253. Oncogenetic testing for persons with hereditary endocrine cancer syndromes

with the following selection of endocrine tumours / syndromes: ? Multiple Endocrine Neoplasia type 1 (MEN1) ? Multiple Endocrine Neoplasia type 2 (MEN2) ? Von Hippel-Lindau (VHL) syndrome ? Phaeochrom ocytoma ? Paraganglioma. Clinicians are encouraged to interpret these recommendations in the context of the individual person/patient situation, values and preferences. All KCE guidelines are as much as possible based on clinical evidence and may not always be in line with the current criteria for NIHDI (RIZIV (...) abnormalities including marfanoid body habitus) ? Familial MTC: family with at least 4 members diagnosed with MTC (in the absence of pheochromocytoma or parathyroid adenoma/hyperplasia)8 Oncogenetic testing in endocrine syndromes KCE Report 242Cs 4.2. Multiple Endocrine Neoplasia type 1 (MEN1) Multiple Endocrine Neoplasia type 1 (MEN1) is a polyglandular genetic syndrome characterized by tumours of the parathyroid glands, pancreatic islet cells and/or anterior pituitary gland. Parathyroid tumours

2015 Belgian Health Care Knowledge Centre

254. A Study to Evaluate the Safety, Tolerability, and Activity of TAK-931 in Participants With Metastatic Pancreatic Cancer, Metastatic Colorectal Cancer, and Other Advanced Solid Tumors

to Brief Summary: The purpose of this study is to confirm the safety and tolerability of TAK-931 in a cohort of Western participants with metastatic solid tumors and to evaluate the anti-tumor activity of TAK-931 in participants with metastatic pancreatic cancer, colorectal cancer (CRC), squamous esophageal cancer (sqEC), and squamous non-small-cell lung cancer (sqNSCLC). Condition or disease Intervention/treatment Phase Metastatic Pancreatic Cancer Colorectal Cancer Esophageal Neoplasms Carcinoma, Non (...) System Neoplasms Neoplasms by Site Neoplasms Digestive System Diseases Gastrointestinal Diseases Colonic Diseases Intestinal Diseases Rectal Diseases Endocrine Gland Neoplasms Pancreatic Diseases Endocrine System Diseases Carcinoma, Bronchogenic Bronchial Neoplasms Lung Neoplasms Respiratory Tract Neoplasms Thoracic Neoplasms Lung Diseases Respiratory Tract Diseases Head and Neck Neoplasms Esophageal Diseases

2017 Clinical Trials

255. APN401 in Treating Patients With Recurrent or Metastatic Pancreatic Cancer, Colorectal Cancer, or Other Solid Tumors That Cannot Be Removed by Surgery

effects and best dose of APN401 in treating patients with pancreatic cancer, colorectal cancer, or other solid tumors that have spread to other places in the body or have come back. APN401 may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth. Condition or disease Intervention/treatment Phase Metastatic Malignant Neoplasm in the Brain Metastatic Solid Neoplasm Recurrent Colorectal Carcinoma Recurrent Pancreatic Carcinoma Recurrent Solid Neoplasm Stage IV Colorectal (...) Cancer Stage IV Pancreatic Cancer Stage IVA Colorectal Cancer Stage IVA Pancreatic Cancer Stage IVB Colorectal Cancer Stage IVB Pancreatic Cancer Unresectable Solid Neoplasm Other: Laboratory Biomarker Analysis Biological: siRNA-transfected Peripheral Blood Mononuclear Cells APN401 Phase 1 Detailed Description: PRIMARY OBJECTIVES: I. To determine the toxicities and establish the safety of multiple infusions of small interfering ribonucleic acid (siRNA)-transfected peripheral blood mononuclear cells

2017 Clinical Trials

256. Long-term results of the surgical management of insulinoma patients with Multiple Endocrine type 1: a groupe d'étude des tumeurs endocrines (GTE) retrospective study. Full Text available with Trip Pro

Long-term results of the surgical management of insulinoma patients with Multiple Endocrine type 1: a groupe d'étude des tumeurs endocrines (GTE) retrospective study. Management of insulinomas in the context of MEN1 remains poorly studied. The aim of this study was to evaluate long-term results of various surgical approaches in a large cohort of insulinoma-MEN1 patients.Consecutive insulinoma-MEN1 patients operated on for a nonmetastatic insulinoma between 1957 and 2010 were retrospectively (...) selected from the MEN1 database of the French Endocrine Tumor Group. The type of surgery was categorized as distal pancreatectomy (DP), total pancreatectomy/cephalic duodenopancreatectomy (TP/CDP), or enucleation (E). Primary endpoint was time until recurrence of hypoglycemia after initial surgery. Secondary endpoints were post-operative complications.The study included 73 patients (median age=28 years). Surgical procedures were DP (n=46), TP/CDP (n=9), or E (n=18). After a median post-operative follow

2014 European Journal of Endocrinology

257. Thymic Epithelial Tumours

ofmyastheniccrisisinspecificsituationssuchasstressortheadministrationofcertaindrugs[V,A]. WHO, World Health Organization; MEN1, multiple endocrine neoplasia 1; CT, computed tomography; MRI, magnetic resonance imaging; PET scan, positron emission tomography scan; AJCC, American Joint Committee on Cancer; UICC, Union for International Cancer Control; OS, overall survival; RECIST, Response Evaluation Criteria in Solid Tumours; IASLC, International Association for the Study of Lung Cancer; ITMIG, International Thymic MalignancyInterestGroup;TNM,tumournodemetastasis. v (...) to play a role in the pathogenesis of thymic epithelial tumours. Reports on development of thymoma after radiation, solid-organ transplantation and immunosuppression, including the context of human immunode?ciency virus infection, are rare; differential diagnosis with thymic rebound hyperplasia maybediscussedinthissetting(seebelow). Genetic risk factors, such as multiple endocrine neoplasia 1 (MEN1), may in?uence the development of thymomas, as well as thymic carcinoids, given their reported familial

2015 European Society for Medical Oncology

258. South Australian gastroenteropancreatic neuroendocrine tumours pathway

of the Minister for Health and Ageing or Department of Health. Other Authors/Contributors: South Australia Department of Health www.sahealth.sa.gov.au Health Information > Cancer Care Pathways. All web links and information live at time of publication. National Library of Australia Cataloguing in Publication entry Title: South Australian Gastroenteropancreatic Neuroendocrine Tumour Pathway ISBN: 978-1-74243-612-8 The South Australian Gastroenteropancreatic Neuroendocrine Tumours Pathway was released in 2013 (...) in the keyword. South Australian GEP NETs Pathway Page 3 of 111 CONTENTS ACKNOWLEDGEMENTS 6 EXECUTIVE SUMMARY 8 KEY RECOMMENDATIONS 10 1. INTRODUCTION 12 1.1 ABOUT CANCER PATHWAYS 12 1.2 INTRODUCTION TO THE SOUTH AUSTRALIAN GASTROENTEROPANCREATIC NEUROENDOCRINE TUMOUR PATHWAY 13 1.3 FURTHER INFORMATION 14 2. GEP NETs IN SOUTH AUSTRALIA 16 2.1 INCIDENCE AND TRENDS 16 2.2 MORTALITY AND SURVIVAL 16 2.3 ETHNIC AND SOCIOECONOMIC DIFFERENCES 17 2.4 FURTHER INFORMATION 18 RECOMMENDATIONS 18 3. MULTIDISCIPLINARY

2014 Clinical Practice Guidelines Portal

259. Defining the role of different endocrine treatment approaches in endocrine-sensitive and endocrine-resistant, advanced, hormone receptor-positive breast cancer: a systematic review and network meta-analysis

Defining the role of different endocrine treatment approaches in endocrine-sensitive and endocrine-resistant, advanced, hormone receptor-positive breast cancer: a systematic review and network meta-analysis 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 (...) will be performed in two phases, namely initial screening based on title 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

2018 PROSPERO

260. Multiple Endocrine Neoplasia, 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

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