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Multiple Endocrine Neoplasia Type 1

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41. Multiple Endocrine Neoplasia and Hyperparathyroid-Jaw Tumor Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood. Full Text available with Trip Pro

Multiple Endocrine Neoplasia and Hyperparathyroid-Jaw Tumor Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood. Children and adolescents who present with neuroendocrine tumors are at extremely high likelihood of having an underlying germline predisposition for the multiple endocrine neoplasia (MEN) syndromes, including MEN1, MEN2A and MEN2B, MEN4, and hyperparathyroid-jaw tumor (HPT-JT) syndromes. Each of these autosomal dominant syndromes results from (...) and location for which specific tumor types most commonly present. Although the recommended surveillance strategies for each syndrome contain similar approaches, important differences do exist among them. Therefore, it is important for caregivers of children and adolescents with these syndromes to become familiar with the unique diagnostic criteria for each syndrome, and also to be aware of the specific tumor screening and prophylactic surgery recommendations for each syndrome. Clin Cancer Res; 23(13

2017 Clinical Cancer Research

42. Clinical Features of Multiple Endocrine Neoplasia Type 4 - Novel pathogenic variant and review of published cases. Full Text available with Trip Pro

presented primarily with primary hyperparathyroidism and functioning and non-functioning pituitary tumors.Hypercalcaemia due to primary hyperparathyroidism and pituitary tumors are common in MEN4. Gastro-intestinal neuroendocrine tumors appear to be less prevalent in MEN4 than in multiple endocrine neoplasia type 1.Copyright © 2019 Endocrine Society. (...) Clinical Features of Multiple Endocrine Neoplasia Type 4 - Novel pathogenic variant and review of published cases. The clinical phenotype of multiple endocrine neoplasia type 4 (MEN4) is undefined due to a limited number of published cases. Knowledge on disease manifestation in MEN4 is essential for developing prevention programs and treatment.To expand current knowledge of the MEN4 phenotype including assessment of penetrance.This is a case report and a brief review of previously published

2019 Journal of Clinical Endocrinology and Metabolism

43. Autologous Tumor Infiltrating Lymphocytes MDA-TIL in Treating Patients With Recurrent or Refractory Ovarian Cancer, Osteosarcoma, or Pancreatic Ductal Adenocarcinoma

Program) ) 2017-0671 ( Other Identifier: M D Anderson Cancer Center ) First Posted: August 1, 2018 Last Update Posted: March 22, 2019 Last Verified: March 2019 Layout table for additional information Studies a U.S. FDA-regulated Drug Product: Yes Studies a U.S. FDA-regulated Device Product: No Product Manufactured in and Exported from the U.S.: No Additional relevant MeSH terms: Layout table for MeSH terms Carcinoma Adenocarcinoma Osteosarcoma Ovarian Neoplasms Carcinoma, Ovarian Epithelial (...) Cystadenocarcinoma, Serous Carcinosarcoma Mixed Tumor, Mullerian Neoplasms, Glandular and Epithelial Neoplasms by Histologic Type Neoplasms Neoplasms, Bone Tissue Neoplasms, Connective Tissue Neoplasms, Connective and Soft Tissue Sarcoma Endocrine Gland Neoplasms Neoplasms by Site Ovarian Diseases Adnexal Diseases Genital Diseases, Female Genital Neoplasms, Female Urogenital Neoplasms Endocrine System Diseases Gonadal Disorders Cystadenocarcinoma Neoplasms, Cystic, Mucinous, and Serous Neoplasms, Complex

2018 Clinical Trials

44. Management of Appendiceal Neoplasms

= peritoneal mucinous carcinomatosis; PMCA-S = peritoneal mucinous carcinomatosis with signet ring cells; PMP = pseudomyxoma peritonei.Copyright © The American Society of Colon & Rectal Surgeons, Inc. Unauthorized reproduction of this article is prohibited. DISEASES OF THE COLON & RECTUM VOLUME 62: 12 (2019) 1427 AND (adenocarcinoma, carcinoma, mucinous, pseudomyx- oma, signet*, cystadenoma, tumor*, tumour*, neoplasm, cancer). These groups were combined with various treat- ment modalities to include (...) . Final recommendations were approved by the ASCRS Executive Committee. In general, Primary search terms: (appendiceal OR appendix OR appendicular) AND (adenocarcinoma OR carcinoma OR mucinous OR pseudomyxoma OR signet* OR cystadenoma OR tumor* OR tumour* OR neoplasm* OR cancer*) AND (therapy OR treatment OR chemotherapy OR HIPEC OR immunotherapy OR antineoplastic OR surgery OR oncological procedure OR diagnosis) Databases: Ovid Medline, Embase, Scopus Years covered: January 1, 1997–April 30, 2019

2020 American Society of Colon and Rectal Surgeons

45. Limited Value of Ga-68-DOTATOC-PET-CT in Routine Screening of Patients with Multiple Endocrine Neoplasia Type 1. (Abstract)

Limited Value of Ga-68-DOTATOC-PET-CT in Routine Screening of Patients with Multiple Endocrine Neoplasia Type 1. Routine screening is recommended for patients with multiple endocrine neoplasia type 1 (MEN1) to enable early detection and treatment of associated neuroendocrine neoplasms (NEN). Gallium68-DOTATOC-Positron emission tomography combined with computed tomography (Ga-68-DOTATOC-PET-CT) is a very sensitive and specific imaging technique for the detection of sporadic neuroendocrine tumors (...) -PET-CT detected more liver and lymph node metastases in patients with known metastatic disease, which did not lead to a change of patients' management. In one patient (3%), Ga-68-DOTATOC-PET-CT was the only imaging modality that detected a small intestine NEN and led to potentially curative surgery.Ga-68-DOTATOC-PET-CT cannot be recommended for routine screening of MEN1 patients. It might provide important additional information in patients with suspected or known metastatic disease.

2017 World Journal of Surgery

46. Impact of "Tailored" Parathyroidectomy for Treatment of Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1. (Abstract)

Impact of "Tailored" Parathyroidectomy for Treatment of Primary Hyperparathyroidism in Patients with Multiple Endocrine Neoplasia Type 1. Whether total parathyroidectomy (TPTX) or subtotal parathyroidectomy (SPTX) should be performed for primary hyperparathyroidism (PHPT) in patients with multiple endocrine neoplasia type 1 (MEN1) is controversial. At our institution, the parathyroidectomy strategy is based on the number of enlarged intraoperative parathyroid glands. We retrospectively analyzed (...) rate based on Kaplan-Meier analysis for each type of surgical procedure.Forty-five patients were analyzed. The overall 5- and 10-year DFS was 91.7 and 55.8%, respectively. The 5- and 10-year DFS in each subgroup was 100.0 and 85.7% in the TPTX group, 89.4 and 57.3% in the SPTX group, and 91.6 and 57.3% in the LPTX group, respectively. The postoperative calcium replacement rate at 1 and 12 months was 91.7 and 58.3% in the TPTX group, 21.1 and 7.0% in the SPTX group, and 30.0 and 0.0% in the LPTX

2017 World Journal of Surgery

47. Study and Follow-up of Multiple Endocrine Neoplasia Type 1

-regulated Device Product: No Additional relevant MeSH terms: Layout table for MeSH terms Neoplasms Multiple Endocrine Neoplasia Endocrine Gland Neoplasms Multiple Endocrine Neoplasia Type 1 Neoplasms by Site Neoplasms, Multiple Primary Neoplastic Syndromes, Hereditary Genetic Diseases, Inborn Endocrine System Diseases (...) Hospitalier Universitaire Dijon Information provided by (Responsible Party): Centre Hospitalier Universitaire Dijon Study Details Study Description Go to Brief Summary: Multiple Endocrine Neoplasia type I (MEN1) or Wermer syndrome is an autosomal dominant disease that predisposes patients to the development of endocrine tumours, principally parathyroid, pituitary or duodenal-pancreatic tumours. It is due to mutations that abolish the function of the MEN1 gene, which contributes to tumour regulation

2017 Clinical Trials

48. Metastatic multiple endocrine neoplasia type 1: report of one case Full Text available with Trip Pro

Metastatic multiple endocrine neoplasia type 1: report of one case A 46-year-old Chinese woman was admitted to our hospital because of presence of space-occupying lesions in the liver for 2 months in April, 2015. She had a family history of multiple endocrine neoplasia type 1 (MEN1) and physical examination is unremarkable. Previously, she has performed surgery for primary pituitary tumor in 2002 and radiosurgery for its recurrence. Around December 2014, she suffered from abdominal discomfort (...) an elevated serum chromogranin A (CgA) level. There was medical evidence to show that she is metastatic MEN1. Although multiple liver metastases, they were considered to be resectable after MDT consultation. The intraoperative exploration found a 1.5 cm tumor on the surface of the tail of the pancreas, a 12 cm retroperitoneal lipoma and two liver metastases, sized 3.5 cm and 1.5 cm, respectively. All these tumors were completely removed. For pancreatic tumor, pathological findings met the diagnostic

2016 Translational gastroenterology and hepatology

49. A novel MEN1 mutation in a Japanese adolescent with multiple endocrine neoplasia type 1 Full Text available with Trip Pro

A novel MEN1 mutation in a Japanese adolescent with multiple endocrine neoplasia type 1 28203045 2018 11 13 0918-5739 26 1 2017 Jan Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology Clin Pediatr Endocrinol A novel MEN1 mutation in a Japanese adolescent with multiple endocrine neoplasia type 1. 25-28 10.1297/cpe.26.25 Itoh Masatsune M Department of Pediatrics, Kanazawa Medical University, Ishikawa (...) , Japan. Saikawa Yutaka Y Department of Pediatrics, Kanazawa Medical University, Ishikawa, Japan. eng Journal Article 2017 01 31 Japan Clin Pediatr Endocrinol 9433330 0918-5739 adolescent missense mutation multiple endocrine neoplasia type 1 (MEN1) 2016 05 19 2016 09 16 2017 2 17 6 0 2017 2 17 6 0 2017 2 17 6 1 ppublish 28203045 10.1297/cpe.26.25 2016-0015 PMC5295248 Clin Endocrinol (Oxf). 2012 Apr;76(4):533-9 21950691 Endocr J. 2012;59(10):859-66 22785103 Mol Cell Endocrinol. 2014 Apr 5;386(1-2):2-15

2017 Clinical Pediatric Endocrinology

50. Genetic confirmation that ependymoma can arise as part of multiple endocrine neoplasia type 1 (MEN1) syndrome Full Text available with Trip Pro

Cancer Genomics Laboratory, University of California, San Francisco, CA, USA. david.solomon@ucsf.edu. eng DP5 OD021403 OD NIH HHS United States Case Reports Letter Research Support, N.I.H., Extramural 2017 02 25 Germany Acta Neuropathol 0412041 0001-6322 0 MEN1 protein, human 0 Proto-Oncogene Proteins IM Adult Ependymoma complications diagnostic imaging genetics Genetic Testing Humans Magnetic Resonance Imaging Male Multiple Endocrine Neoplasia Type 1 complications diagnostic imaging genetics (...) Genetic confirmation that ependymoma can arise as part of multiple endocrine neoplasia type 1 (MEN1) syndrome 28238068 2018 05 31 2018 11 13 1432-0533 133 4 2017 04 Acta neuropathologica Acta Neuropathol. Genetic confirmation that ependymoma can arise as part of multiple endocrine neoplasia type 1 (MEN1) syndrome. 661-663 10.1007/s00401-017-1689-7 Cuevas-Ocampo Areli K AK Division of Neuropathology, Department of Pathology, University of California, San Francisco, 513 Parnassus Ave, Health

2017 Acta neuropathologica

51. Radiological surveillance in multiple endocrine neoplasia type 1: a double-edged sword? Full Text available with Trip Pro

Radiological surveillance in multiple endocrine neoplasia type 1: a double-edged sword? Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition characterised by the predisposition to hyperplasia/tumours of endocrine glands. MEN1-related disease, moreover, malignancy related to MEN1, is increasingly responsible for death in up to two-thirds of patients. Although patients undergo radiological and biochemical surveillance, current recommendations for radiological monitoring are based (...) obtained details regarding all radiological procedures performed as part of MEN1 surveillance or disease localisation. An estimated effective radiation dose (ED) for each individual patient was calculated.The mean ED for the total patient cohort was 121 mSv, and the estimated mean lifetime risk of cancer secondary to radiation exposure was 0.49%. Patients with malignant neuroendocrine tumours (NETS) had significantly higher ED levels compared to patients without metastatic disease (P < 0.0022).In MEN1

2017 Endocrine connections

52. Coexistence of GH-Producing Pituitary Macroadenoma and Meningioma in a Patient with Multiple Endocrine Neoplasia Type 1 with Hyperglycemia and Ketosis as First Clinical Sign Full Text available with Trip Pro

Coexistence of GH-Producing Pituitary Macroadenoma and Meningioma in a Patient with Multiple Endocrine Neoplasia Type 1 with Hyperglycemia and Ketosis as First Clinical Sign We present the clinical case of a patient who was admitted with an onset of diabetes mellitus (DM) with associated ketosis and whose clinical, hormonal, and radiological evolution revealed the presence of primary hyperparathyroidism, pancreatic neuroendocrine tumor, and GH-producing pituitary macroadenoma in the context (...) of multiple endocrine neoplasia type 1 (MEN1). DM is relatively common in cases of acromegaly, but it is not generally associated with ketosis. Simultaneously, the patient presented a meningioma, which is associated with pituitary macroadenoma only in extremely rare cases.

2017 Case reports in endocrinology

53. The LARO-MEN1 study: a longitudinal clinical experience with octreotide Long-Acting Release in patients with Multiple Endocrine Neoplasia type 1 Syndrome Full Text available with Trip Pro

The LARO-MEN1 study: a longitudinal clinical experience with octreotide Long-Acting Release in patients with Multiple Endocrine Neoplasia type 1 Syndrome Multiple endocrine neoplasia type 1 (MEN1) is a rare hereditary tumoral syndrome, featured by a combination of neoplasms of various endocrine and nonendocrine tissues. Approximately 33% of MEN1-related deaths are due to the malignant behaviour of well-differentiated neuroendocrine tumors (NETs), for which a preventive surgical treatment (...) is not feasible. Somatostatin analogues (SSA) have been employed in the treatment of NETs in the stage of advanced or metastatic disease, in order to control the growth and secretion of tumor lesions. A longitudinal, open label study named "LARO-MEN1" was undertaken in order to assess whether early medical treatment with long-acting SSA could act as a preventive approach in small MEN1-related gastroenteropancreatic (GEP) NETs. Thirty consecutive patients affected by MEN1 were screened and 8 patients

2017 Clinical Cases in Mineral and Bone Metabolism

54. Challenging Differential Diagnosis of Hypergastremia and Hyperglucagonemia with Chronic Renal Failure: Report of a Case with Multiple Endocrine Neoplasia Type 1 Full Text available with Trip Pro

Challenging Differential Diagnosis of Hypergastremia and Hyperglucagonemia with Chronic Renal Failure: Report of a Case with Multiple Endocrine Neoplasia Type 1 A 53-year-old woman developed end-stage renal failure during a 15-year clinical course of primary hyperparathyroidism and was referred to our hospital for evaluation of suspected multiple endocrine neoplasia type 1 (MEN1). Genetic testing revealed a novel deletion mutation at codon 467 in exon 10 of the MEN1 gene. Systemic and selective

2017 Internal Medicine

55. Genetics of multiple endocrine neoplasia type 1 syndrome: what's new and what's old Full Text available with Trip Pro

Genetics of multiple endocrine neoplasia type 1 syndrome: what's new and what's old Despite its identification in 1997, the functions of the MEN1 gene-the main gene underlying multiple endocrine neoplasia type 1 syndrome-are not yet fully understood. In addition, unlike the RET-MEN2 causative gene-no hot-spot mutational areas or genotype-phenotype correlations have been identified. More than 1,300 MEN1 gene mutations have been reported and are mostly "private" (family specific). Even when (...) mutations are shared at an intra- or inter-familial level, the spectrum of clinical presentation is highly variable, even in identical twins. Despite these inherent limitations for genetic counseling, identifying MEN1 mutations in individual carriers offers them the opportunity to have lifelong clinical surveillance schemes aimed at revealing MEN1-associated tumors and lesions, dictates the timing and scope of surgical procedures, and facilitates specific mutation analysis of relatives to define

2017 F1000Research

56. Subtotal versus total parathyroidectomy for primary hyperparathyroidism in multiple endocrine neoplasia type 1: a systematic review update

Subtotal versus total parathyroidectomy for primary hyperparathyroidism in multiple endocrine neoplasia type 1: a systematic review update 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. The registrant confirms that the information supplied for this submission is accurate and complete. CRD bears no responsibility or liability for the content of this registration (...) criteria: Example: Screening 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

2019 PROSPERO

57. A case of type 1 multiple endocrine neoplasia with esophageal stricture successfully treated with endoscopic balloon dilation and local steroid injection combined with surgical resection of gastrinomas. Full Text available with Trip Pro

A case of type 1 multiple endocrine neoplasia with esophageal stricture successfully treated with endoscopic balloon dilation and local steroid injection combined with surgical resection of gastrinomas. In type 1 multiple endocrine neoplasia (MEN1), esophageal diseases association with excessive gastrin secretion in Zollinger-Ellison syndrome (ZES) sometimes develop. Here, we reported a case of MEN1/ZES, who developed dysphagia due to reflux esophagitis with severe esophageal stricture (...) . Treatment for his esophageal stricture and ZES was discussed.A 43-year-old man with progressive dysphagia and diarrhea was referred to the teaching hospital. He had a history of recurrent duodenojejunal perforations despite the anti-secretory medication. Blood examinations revealed elevated serum gastrin, calcium, and parathyroid hormone. Upper gastrointestinal endoscopy demonstrated a severe esophageal stricture, multiple gastroduodenal ulcer scars, and a duodenal submucosal tumor. Enhanced computed

2017 BMC Gastroenterology

58. No Association of Blood Type O with Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1. Full Text available with Trip Pro

No Association of Blood Type O with Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1. An association between ABO blood type and the development of cancer, in particular, pancreatic cancer, has been reported in the literature. An association between blood type O and neuroendocrine tumors in multiple endocrine neoplasia type 1 (MEN1) patients was recently suggested. Therefore, blood type O was proposed as an additional factor to personalize screening criteria for neuroendocrine tumors (...) cohorts (Grays's test for equality; P = 0.72). Furthermore, we found no association between blood type O and the occurrence of metastatic disease or survival.An association between blood type O and the occurrence of neuroendocrine tumors in MEN1 patients was not confirmed. For this reason, the addition of the blood type to screening and surveillance practice seems not to be of additional value for identifying MEN1 patients at risk for the development of neuroendocrine tumors, metastatic disease

2015 Journal of Clinical Endocrinology and Metabolism

59. Growth rate of small pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: results from an endoscopic ultrasound based cohort study. Full Text available with Trip Pro

Growth rate of small pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: results from an endoscopic ultrasound based cohort study. Background and aims In multiple endocrine neoplasia type 1 (MEN1), endoscopic ultrasound (EUS) is used for identification and follow-up of pancreatic neuroendocrine tumors (PNETs). The role of EUS in surveillance of small ( < 20 mm) PNETs is unclear, mostly because the natural course of these lesions is largely unknown. We aimed to determine

2016 Endoscopy

60. Utility of chromogranin A, pancreatic polypeptide, glucagon, and gastrin in the diagnosis and follow-up of pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 patients Full Text available with Trip Pro

Utility of chromogranin A, pancreatic polypeptide, glucagon, and gastrin in the diagnosis and follow-up of pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1 patients Pancreatic neuroendocrine tumours (PNETs) are the major source of disease-specific mortality in multiple endocrine neoplasia type 1 (MEN1) patients. Chromogranin A (CgA), pancreatic polypeptide (PP), glucagon and gastrin have some diagnostic value in sporadic PNETs, but there is very little evidence (...) MEN1 cases, 55 PNETs and 58 non-PNETs met inclusion criteria. The area under the curve (AUC) for CgA, PP, glucagon and gastrin in MEN1 cases was 59·5%, 64·1%, 77·0% and 75·9%, respectively. The AUC for the combination of CgA, PP and gastrin was 59·6%. PP, but not CgA, glucagon or gastrin was significantly associated with both age and PNET functional status (P = 0·0485 and 0·0188, respectively). No markers were significantly associated with sex, PNET size, tumour number, tumour location, American

2016 Clinical endocrinology

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