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https://www.readbyqxmd.com/read/28810315/-adult-peripheral-primary-neuroectodermal-tumor-a-case-report-and-literature-review
#1
M H Hu, F Long, S J Jiang
Objective: To analyze the clinical features, diagnosis and differential diagnosis of peripheral primary neuroectodermal tumor(pPNET). Methods: The clinical data and diagnosis of a patient with rapid progressive pPNET in Shandong Provincial Hospital affiliated to Shandong University in January 2016 was reported and the related literatures were reviewed.The literature reviews were carried out respectively in CNKI, Wanfang and PubMed by July 2016 with "primitive neurotodermal tumour" and "PNET" being the search term from March 1994 to July 2016, including 13 articles...
August 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28801375/primary-pure-endometrial-primitive-neuroectodermal-tumors-with-ewsr1-gene-rearrangement-report-of-2-cases
#2
Binara Assylbekova, Amal Kanbour-Shakir, Kavita Varma, Rohit Bhargava, Esther Elishaev
Primary Primitive Neuroectodermal Tumor (PNET) of the female genital tract is very rare. The majority has been described in ovaries, and the endometrium is an even rarer site. Herein we describe 2 cases of primary uterine corpus PNET cases with EWSR1 FISH confirmation. To the best of our knowledge, there are four cases reported in the English literature. The knowledge of these tumors' incidence in the endometrium is important to keep in mind, to differentiate it from poorly differentiated and undifferentiated adenocarcinomas, should such pattern of growth be encountered...
August 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28794363/renal-cell-carcinoma-and-a-pancreatic-neuroendocrine-tumor-a-coincidence-or-instance-of-von-hippel-lindau-disease
#3
Hiroyuki Matsubayashi, Masashi Niwakawa, Katsuhiko Uesaka, Keiko Sasaki, Yoshimi Kiyozumi, Hirotoshi Ishiwatari, Kinichi Hotta, Kenichiro Imai, Sayo Ito, Kohei Takizawa, Masaki Tanaka, Noboru Kawata, Naomi Kakushima, Hiroyuki Ono
We herein report a rare case of a 79-year-old man who presented with the simultaneous occurrence of pancreatic neuroendocrine tumors (PNET) and renal cell carcinomas (RCC), without any other Von Hippel-Lindau (VHL)-associated lesions or any pertinent family history. Computed tomography showed vascular-rich solid lesions in the left kidney and the pancreatic tail, measuring 72 mm and 15 mm in size, respectively. Preoperatively, RCC with pancreatic metastasis was suspected and laparotomy was performed. However, the resected specimens revealed a different tumor histology, namely renal clear cell carcinoma (G2, pT3) and PNET (G1, pT3)...
August 10, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28792692/comparison-of-tumor-markers-for-predicting-disease-free-survival-in-surgically-resected-pancreatic-neuroendocrine-tumors
#4
Bo Zhou, Bingliang Fang, Sheng Yan, Weilin Wang
BACKGROUND: Identification of biomarkers of pancreatic neuroendocrine tumors (PNETs) is important for stratification of the prognosis. Ki-67 index was the significant prognostic factor for PNETs. Recently, MMP-9, DJ-1, and α-1-B glycoprotein (A1BG) were shown to be the prognostic markers in some malignant tumors except for PNETs. The aim of this study was to compare these tumor markers for predicting disease-free survival in surgically resected PNETs. METHODS: A retrospective review of patients pathologically diagnosed with PNETs at our institution from January 2012 to January 2014 was conducted...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28766979/pancreatic-neuroendocrine-tumor-prediction-of-the-tumor-grade-using-ct-findings-and-computerized-texture-analysis
#5
Tae Won Choi, Jung Hoon Kim, Mi Hye Yu, Sang Joon Park, Joon Koo Han
Background Pancreatic neuroendocrine tumors (PNET) include heterogeneous tumors with a variable degree of inherent biologic aggressiveness represented by the histopathologic grade. Although several studies investigated the computed tomography (CT) characteristics which can predict the histopathologic grade of PNET, accurate prediction of the PNET grade by CT examination alone is still limited. Purpose To investigate the important CT findings and CT texture variables for prediction of grade of PNET. Material and Methods Sixty-six patients with pathologically confirmed PNETs (grade 1 = 45, grades 2/3 = 21) underwent preoperative contrast-enhanced CT...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28765340/lack-of-association-for-reported-endocrine-pancreatic-cancer-risk-loci-in-the-pandora-consortium
#6
Daniele Campa, Ofure Obazee, Manuela Pastore, Francesco Panzuto, Valbona Liço, William Greenhalf, Verena Katzke, Francesca Tavano, Eithne Costello, Vincenzo Corbo, Renata Talar-Wojnarowska, Oliver Strobel, Carlo Federico Zambon, John P Neoptolemos, Giulia Zerboni, Rudolf Kaaks, Timothy J Key, Carlo Lombardo, Krzysztof Jamroziak, Domenica Gioffreda, Thilo Hackert, Kay-Tee Khaw, Stefano Landi, Anna Caterina Milanetto, Luca Landoni, Rita T Lawlor, Franco Bambi, Felice Pirozzi, Daniela Basso, Claudio Pasquali, Gabriele Capurso, Federico Canzian
Background: Pancreatic neuroendocrine tumors (PNETs) are rare neoplasms for which very little is known about either environmental or genetic risk factors. Only a handful of association studies have been performed so far, suggesting a small number of risk loci.Methods: To replicate the best findings, we have selected 16 SNPs suggested in previous studies to be relevant in PNET etiogenesis. We genotyped the selected SNPs (rs16944, rs1052536, rs1059293, rs1136410, rs1143634, rs2069762, rs2236302, rs2387632, rs3212961, rs3734299, rs3803258, rs4962081, rs7234941, rs7243091, rs12957119, and rs1800629) in 344 PNET sporadic cases and 2,721 controls in the context of the PANcreatic Disease ReseArch (PANDoRA) consortium...
August 2017: Cancer Epidemiology, Biomarkers & Prevention
https://www.readbyqxmd.com/read/28763801/surgical-resection-of-primary-tumor-improves-survival-of-pancreatic-neuroendocrine-tumor-with-liver-metastases
#7
Tao Lianyuan, Xiu Dianrong, Abuduhaibaier Sadula, Ye Chen, Chen Qing, Wang Hanyan, Zhang Zhipeng, Zhang Lingfu, Tao Ming, Yuan Chunhui
This study investigates survival of patients diagnosed with pancreatic neuroendocrine tumor with liver metastases based on local treatment on the primary tumor. Patients diagnosed with stage IV PNET between 2010 and 2014 were identified from the Surveillance Epidemiology and End Results database. Cancer-Specific Survival and Overall Survival were examined. A total of 191 patients with pancreatic neuroendocrine tumor with liver metastases were included in this analysis. There were 47 patients (24.6%) who received surgical resection and 144 (75...
July 24, 2017: Oncotarget
https://www.readbyqxmd.com/read/28761507/primary-intracranial-dural-based-ewing-sarcoma-peripheral-primitive-neuroectodermal-tumor-mimicking-a-meningioma-a-rare-tumor-with-review-of-literature
#8
REVIEW
Vikul Kumar, Anshu Singh, Vivek Sharma, Mohan Kumar
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is a malignant small, round cell tumor arising from bone and soft tissue in children and young adults. It can occur at osseous and extraosseous sites. Its usual locations are diaphysis of long bones followed by pelvis, ribs, vertebrae, and rarely skull. We reviewed the literature and PubMed advanced search on ES/pPNET occurring at extraosseous sites, mainly involving the central nervous system (CNS). We reported a case of a 22-year-old male presenting with seizure finally diagnosed as a case of ES/pPNET...
July 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28753586/development-of-zebrafish-medulloblastoma-like-pnet-model-by-talen-mediated-somatic-gene-inactivation
#9
Jaegal Shim, Jung-Hwa Choi, Moon-Hak Park, Hyena Kim, Jong Hwan Kim, Seon-Young Kim, Dongwan Hong, Sunshin Kim, Ji Eun Lee, Cheol-Hee Kim, Jeong-Soo Lee, Young-Ki Bae
Genetically engineered animal tumor models have traditionally been generated by the gain of single or multiple oncogenes or the loss of tumor suppressor genes; however, the development of live animal models has been difficult given that cancer phenotypes are generally induced by somatic mutation rather than by germline genetic inactivation. In this study, we developed somatically mutated tumor models using TALEN-mediated somatic gene inactivation of cdkn2a/b or rb1 tumor suppressor genes in zebrafish. One-cell stage injection of cdkn2a/b-TALEN mRNA resulted in malignant peripheral nerve sheath tumors with high frequency (about 39%) and early onset (about 35 weeks of age) in F0 tp53e7/e7 mutant zebrafish...
July 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28744737/neuroendocrine-liver-metastasis-prognostic-implications-of-primary-tumor-site-on-patients-undergoing-curative-intent-liver-surgery
#10
Gaya Spolverato, Fabio Bagante, Luca Aldrighetti, George Poultsides, Todd W Bauer, Ryan C Field, Hugo P Marques, Matthew Weiss, Shishir K Maithel, Timothy M Pawlik
BACKGROUND: Neuroendocrine tumors typically arise from pancreatic (PNET) vs. gastrointestinal or thoracic origins (non-PNET). The impact of primary tumor site on long-term prognosis following resection of neuroendocrine liver metastasis (NELM) remains poorly defined. The objective of the current study was to define the association of primary tumor location on prognosis of patients undergoing curative intent liver resection for NELM. METHODS: Between 1990 and 2014, 421 patients who underwent resection of NELM were identified from a multi-institutional database...
July 25, 2017: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/28744687/lin28a-a-sensitive-immunohistochemical-marker-for-embryonal-tumor-with-multilayered-rosettes-etmr-is-also-positive-in-a-subset-of-atypical-teratoid-rhabdoid-tumor-at-rt
#11
Shilpa Rao, R T Rajeswarie, T Chickabasaviah Yasha, Bevinahalli N Nandeesh, Arimappamagan Arivazhagan, Vani Santosh
INTRODUCTION: CNS embryonal tumors comprise a group of highly malignant neoplasms with a wide spectrum of histomorphological entities that includes Medulloblastoma (MB), Atypical Teratoid/Rhabdoid Tumor (AT/RT), Neuroblastoma (NB), Ganglioneuroblastoma (GNB), Embryonal Tumor with Multilayered Rosettes (ETMR), and the embryonal tumor-Not Otherwise Specified (NOS). The entity ETMR includes previously described histopathologic patterns-Embryonal Tumor with Abundant Neuropil and True Rosettes (ETANTR), Ependymoblastoma (EBL), and Medulloepithelioma (MEPL)...
July 25, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28736585/late-onset-asymptomatic-pancreatic-neuroendocrine-tumor-a-case-report-on-the-phenotypic-expansion-for-men1
#12
Charu Kaiwar, Sarah K Macklin, Jennifer M Gass, Jessica Jackson, Eric W Klee, Stephanie L Hines, John A Stauffer, Paldeep S Atwal
BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a hereditary cancer syndrome associated with several endocrine as well as non-endocrine tumors and is caused by mutations in the MEN1 gene. Primary hyperparathyroidism affects the majority of MEN1 individuals by age 50 years. Additionally, MEN1 mutations trigger familial isolated hyperparathyroidism. We describe a seemingly unaffected 76-year-old female who presented to our Genetics Clinic with a family history of primary hyperparathyroidism and the identification of a pathogenic MEN1 variant...
2017: Hereditary Cancer in Clinical Practice
https://www.readbyqxmd.com/read/28730672/lipid-lowering-therapy-of-everolimus-related-severe-hypertriglyceridaemia-in-a-pancreatic-neuroendocrine-tumour-pnet
#13
V De Gennaro Colonna, C Pavanello, F Rusconi, A Sartore-Bianchi, S Siena, S Castelnuovo, C R Sirtori, G Mombelli
WHAT IS KNOWN AND OBJECTIVE: Hypertriglyceridaemia (HTG) is a potentially serious side effect of everolimus therapy. We here report a case of severe HTG in an everolimus-treated patient and provide recommendations for its management. CASE SUMMARY: The patient was a 70-year-old woman, being treated with everolimus for a pancreatic neuroendocrine tumour (pNET). She developed severe HTG to a maximum of 969 mg/dL after 22 months of therapy. Treatment with fenofibrate rapidly normalized triglyceride (TG) levels...
July 21, 2017: Journal of Clinical Pharmacy and Therapeutics
https://www.readbyqxmd.com/read/28728220/-diagnostic-utility-of-tyrosine-hydroxylase-in-peripheral-neuroblastic-tumors
#14
N Zhang, N Liu, L J He
Objective: To investigation the diagnostic utility of tyrosine hydroxylase (TH) immunohistochemically as a marker of peripheral neuroblastic tumors(pNT). Methods: The study included 1 024 cases, 643 primary and metastatic pNT cases, 381 non-pNT cases, including small round cell tumors such as primitive neuroectodermal tumor (PNET), rhabdomyosarcoma, lymphoma, nephroblastoma, as well as other more common tumors (medulloblastoma, hepatoblastoma, pleuropulmonary blastoma, renal clear cell sarcoma, Langerhans cell histiocytosis, lipoblatoma etc)...
July 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/28728148/the-role-of-cytotoxic-chemotherapy-in-advanced-pancreatic-neuroendocrine-tumors
#15
Sebastian Krug, Thomas M Gress, Patrick Michl, Anja Rinke
BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) are rare neoplasms accounting for less than 5% of all pancreatic malignancies. These tumors are characterized by clinical and prognostical heterogeneity and are predominantly diagnosed in a metastatic stage. Cytotoxic chemotherapy, along with alkylating agents and antimetabolites as well as molecular targeted agents (everolimus, sunitinib), is used in the treatment of advanced PNETs. After the approval of lanreotide for unresectable PNETs, an additional therapeutic option has become available; however, the best sequence of therapies and patient stratification to different treatments remains challenging...
July 21, 2017: Digestion
https://www.readbyqxmd.com/read/28724304/synchronous-intraductal-papillary-mucinous-neoplasm-and-a-pancreatic-neuroendocrine-tumor-more-than-a-coincidence
#16
Juliana M Costa, Sofia Carvalho, João B Soares
Although the association between intraductal papillary mucinous neoplasm of the pancreas (IPMN) and pancreatic neuroendocrine tumor (PNET) has been increasingly reported, whether this association is real or coincidence remains unclear. We report a case of synchronous IPMN and a PNET which were diagnosed preoperatively and discuss the tumorigenesis, clinicopathological features and management of these rare tumors based on the published literature. A 56-year-old male was incidentally diagnosed with a 14 mm branch duct IPMN and a 3...
July 14, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28723135/development-of-dual-aptamers-for-constructing-sandwich-type-pancreatic-polypeptide-assay
#17
Shiya Qin, Nandi Chen, Xiaohai Yang, Qing Wang, Kemin Wang, Jin Huang, Jianbo Liu, Maogui Zhou
Pancreatic polypeptide (PP) is a specific biomarker of nonfunctional pancreatic neuroendocrine tumors (NF-pNETs). Clinical significance of PP inspires researchers to make great efforts in developing sensitive and specific sensors. However, there is no existing biosensor for detecting PP that combines facility and functionality. Addressing this challenge, a pair of aptamers which could be used to develop a sandwich assay for PP is reported. First, several high affinity aptamers are screened through graphene oxide-based SELEX, and appropriate dual-aptamers which could bind to different epitopes of PP are identified through fluorescence assays...
February 24, 2017: ACS Sensors
https://www.readbyqxmd.com/read/28718084/pancreatic-neuroendocrine-tumor-producing-insulin-and-vasopressin
#18
Omalkhaire M Alshaikh, Ju-Yoon Yoon, Bryan A Chan, Monika K Krzyzanowska, Jagdish Butany, Sylvia L Asa, Shereen Ezzat
The objective of the study is to report a rare case of pancreatic neuroendocrine tumor (pNET) producing insulin and vasopressin. We describe the clinical presentation and management of a metastatic pNET with refractory hypoglycemia and progressive severe hyponatremia. A 52-year-old patient had abdominal pain leading to the diagnosis of a tumor that was initially presumed to be splenic in origin. Investigations ultimately identified a pancreatic mass that on biopsy proved to be a pNET. Eventually, he developed extensive liver metastases, and with tumor progression, he manifested hypoglycemia and severe hyponatremia...
July 17, 2017: Endocrine Pathology
https://www.readbyqxmd.com/read/28707013/sunitinib-is-effective-and-tolerable-in-chinese-patients-with-advanced-pancreatic-neuroendocrine-tumors-a-multicenter-retrospective-study-in-china
#19
Yuhong Wang, Kaizhou Jin, Huangying Tan, Pan Zhang, Qiuchen Yang, Wei Wang, Jie Li, Chenghao Shao, Ling Xue, Shiting Feng, Minhu Chen, Xianjun Yu, Jie Chen
PURPOSE: To determine the efficacy and safety of sunitinib in Chinese patients with unresectable or metastatic pancreatic neuroendocrine tumors (pNETs) and the clinical significance of steady-state sunitinib serum concentrations. METHODS: We conducted a multicenter retrospective study including six centers from across China. A total of 60 patients with unresectable or metastatic pNETs who were treated with sunitinib were evaluated retrospectively. RESULTS: The median overall survival (OS) was 47...
July 13, 2017: Cancer Chemotherapy and Pharmacology
https://www.readbyqxmd.com/read/28706137/assessment-of-pancreatic-neuroendocrine-tumor-cytologic-genotype-diversity-to-guide-personalized-medicine-using-a-custom-gastroenteropancreatic-next-generation-sequencing-panel
#20
Ferga C Gleeson, Jesse S Voss, Benjamin R Kipp, Sarah E Kerr, John S Van Arnam, John R Mills, Cherisse A Marcou, Amber R Schneider, Zheng Jin Tu, Michael R Henry, Michael J Levy
BACKGROUND: Recent genetic studies have highlighted that alterations in MEN1, chromatin remodeling genes, and mammalian target of rapamycin (mTOR) pathway genes are the most frequent molecular events identified in pancreas neuroendocrine tumors (pNETs). The prognostic or predictive impact of these biomarkers and other less frequently observed aberrations, i.e. PTEN, TSC2 and PIK3CA are relatively unknown. The aims of this targeted next generation sequencing (NGS) study were to assess tumor cytology genotype diversity, to survey for potential adverse prognostic biomarkers and the prevalence of mTOR pathway variants...
June 28, 2017: Oncotarget
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