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https://www.readbyqxmd.com/read/28535439/critical-focus-on-mechanisms-of-resistance-and-toxicity-of-m-tor-inhibitors-in-pancreatic-neuroendocrine-tumors
#1
REVIEW
L Antonuzzo, M Del Re, V Barucca, F Spada, G Meoni, G Restante, R Danesi, F Di Costanzo, N Fazio
Pancreatic neuroendocrine tumors (pNETs) are rare neoplasms representing less than 2% of all pancreatic malignancies. The PI3K-AKT-mTOR pathway is often deregulated in pNETs and seems to play a key role in tumorigenesis. Everolimus, an inhibitor of the mTOR pathway, has demonstrated efficacy in the treatment of pNETs. Nevertheless de novo or acquired drug resistance is responsible for disease progression and represents a major obstacle to overcome by clinicians. Blocking the PI3K/AKT/mTOR pathway may cover the supposed main mechanisms of resistance to everolimus...
May 11, 2017: Cancer Treatment Reviews
https://www.readbyqxmd.com/read/28534008/a-comparison-of-pediatric-vs-adult-patients-with-the-ewing-sarcoma-family-of-tumors
#2
Vivek Verma, Kyle A Denniston, Christopher J Lin, Chi Lin
PURPOSE: This study sought to identify differences in clinical characteristics, outcomes, and treatments between adult and pediatric patients with the Ewing sarcoma family of tumors (ESFT). METHODS: By using the Surveillance, Epidemiology, and End Results database from 1983 to 2013, 1,870 patients were analyzed (n = 976 pediatric, n = 894 adult). Between the two groups, demographic, tumor, and treatment characteristics were collated and compared. The chi-square test determined differences in proportions of the variables between groups...
2017: Frontiers in Oncology
https://www.readbyqxmd.com/read/28515922/primary-primitive-neuroectodermal-tumor-of-the-cervix-a-report-of-two-cases-and-review-of-the-literature
#3
Xiaofeng Wang, Yan Gao, Yanning Xu, Yixin Liu, Pengpeng Qu
Peripheral primitive neuroectodermal tumor (PNET) of the cervix uteri is extremely rare. Two cases of PNET of the cervix are presented herein. Two female patients, aged 48 and 43 years, presented with irregular uterine bleeding over the course of 1 year, and increased urinary frequency for 1 month, respectively. On gynecological examination, a mass in the cervix was palpated and a biopsy performed. The findings of the initial biopsy revealed small-cell carcinoma in both patients. Following neoadjuvant chemotherapy and radiotherapy, radical hysterectomy was performed in both patients...
May 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28510792/a-systematic-review-of-proinsulin-secreting-pancreatic-neuroendocrine-tumors
#4
Timothy D Murtha, Beatrice C Lupsa, Sachin Majumdar, Dhanpat Jain, Ronald R Salem
BACKGROUND: Pancreatic neuroendocrine tumors (PNETs) are a heterogeneous group of islet cell-derived neoplasms with a propensity toward hormone production. Among PNETs, proinsulin-secreting tumors (proinsulinomas) are exceedingly rare. The objective of this study is to collect and summarize the existing literature to provide a comprehensive evaluation of this uncommon disease. METHODS: A systematic review was performed to characterize the clinicopathologic features of proinsulinoma...
May 16, 2017: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/28509337/impact-of-induction-chemotherapy-hyperfractionated-accelerated-radiotherapy-and-high-dose-thiotepa-on-brain-volume-loss-and-functional-status-of-children-with-primitive-neuroectodermal-tumour
#5
Elwira Szychot, Kiran Seunarine, Kshitij Mankad, Steffi Thust, Chris Clark, Mark N Gaze, Antony Michalski
BACKGROUND: The introduction of aggressive chemo-radiotherapy regimens has improved overall survival in children with primitive neuroectodermal tumours (PNET). However, these combinations may result in neurotoxicity. Previously reported magnetic resonance imaging abnormalities in children receiving intensive sequential chemotherapy, hyperfractionated accelerated radiotherapy (HART) and high-dose thiotepa prompted us to investigate the degree of brain volume loss and patients' functional status after therapy...
May 16, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28509141/primary-ewing-sarcoma-of-the-kidney-a-case-report-and-treatment-review
#6
Muhammad Sadiq, Iftikhar Ahmad, Jamila Shuja, Khushnaseeb Ahmad
Ewing sarcomas/primitive neuroectodermal tumors (ES/PNET) of the kidney are rarely found high-grade malignant tumors, offering poor prognosis. Although established treatment guidelines for ES of kidney are scarce, a multi-modality treatment approached is typically implemented. Herein, we report a 14-year-old female patient with ES of right kidney. Post-nephrectomy disease recurrence was treated with chemotherapy (i.e., vincristine, doxorubicin and cyclophosphamide); marked reduction in tumor size (i.e., from 18...
April 21, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508928/embryonal-tumors-in-canadian-children-less-than-36-months-of-age-results-from-the-canadian-pediatric-brain-tumor-consortium-cpbtc
#7
E Story, D L Johnston, U Bartels, A S Carret, B Crooks, D D Eisenstat, C Fryer, L Lafay-Cousin, V Larouche, B Wilson, S Zelcer, M Silva, J Brossard, E Bouffet, D L Keene
Embryonal tumors are a heterogeneous group of central nervous system (CNS) tumors whose subgroups have varying incidence and outcome. Despite these differences, they are often grouped as a single entity for study purposes. To date, there are no Canadian multi-institutional studies examining the incidence and outcome of all embryonal subtypes. The current study is an observational study reviewing embryonal tumors in all patients less than 36 months of age diagnosed with a CNS tumor in Canada from 1990 to 2005...
May 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28504695/epigenetic-pathway-inhibitors-represent-potential-drugs-for-treating-pancreatic-and-bronchial-neuroendocrine-tumors
#8
K E Lines, M Stevenson, P Filippakopoulos, S Müller, H E Lockstone, B Wright, S Grozinsky-Glasberg, A B Grossman, S Knapp, D Buck, C Bountra, R V Thakker
Cancer is associated with alterations in epigenetic mechanisms such as histone modifications and methylation of DNA, and inhibitors targeting epigenetic mechanisms represent a novel class of anti-cancer drugs. Neuroendocrine tumors (NETs) of the pancreas (PNETs) and bronchus (BNETs), which may have 5-year survivals of <50% and as low as 5%, respectively, represent targets for such drugs, as >40% of PNETs and ~35% of BNETs have mutations of the multiple endocrine neoplasia type 1 (MEN1) gene, which encodes menin that modifies histones by interacting with histone methyltransferases...
May 15, 2017: Oncogenesis
https://www.readbyqxmd.com/read/28501118/impact-of-chromogranin-a-differentiation-and-mitoses-in-nonfunctional-pancreatic-neuroendocrine-tumors-%C3%A2-2%C3%A2-cm
#9
Katelin A Mirkin, Christopher S Hollenbeak, Joyce Wong
BACKGROUND: Small pancreatic neuroendocrine tumors (PNETs) are a unique subset of pancreatic neoplasms. Chromogranin A (CgA) levels, mitotic rate, and histologic differentiation are often used to characterize PNET behavior. This study evaluates the impact of these factors on survival in patients with PNETs. METHODS: The US National Cancer Data Base (1998-2012) was reviewed for patients with stages I-III, nonfunctional PNETs ≤2 cm. Clinicopathologic characteristics were collected, and univariate and multivariate survival analyses were performed...
May 1, 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28481064/-diagnosis-and-treatment-of-the-tumors-of-the-parotid-region-in-pediatrics-cohort
#10
R Oesterreich, J Udaquiola, P Lobos, J Moldes, D Liberto
INTRODUCTION: Parotid tumors are rare in children and usually have multiple differential diagnoses that require different diagnostic and treatment methods. MATERIAL AND METHODS: Retrospective cohort study of all consecutive pediatric patients with parotid tumors that were treated in Pediatric Surgery Service of Hospital Italiano de Buenos Aires. RESULTS: Twenty-two patients with parotid tumors were included and 72% (n=16) were female patients...
October 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28480609/pancreatic-neuroendocrine-tumor-correlations-between-mri-features-tumor-biology-and-clinical-outcome-after-surgery
#11
Rodrigo Canellas, Grace Lo, Sreejita Bhowmik, Cristina Ferrone, Dushyant Sahani
PURPOSE: To assess which magnetic resonance imaging (MRI) features are associated with pNETs (pancreatic neuroendocrine tumors) grade based on the WHO classification, as well as identify MRI features related to disease progression after surgery. MATERIALS AND METHODS: In this Institutional Review Board (IRB)-approved study, 1.5T and 3.0T MRI scans of 80 patients with surgically verified pNETs were assessed. The images were evaluated for tumor location; size; pattern; predominant signal intensity on precontrast T1 - and T2 -weighted images, as well as on postcontrast arterial and portal venous phase T1 -weighted sequences; presence of pancreatic duct dilatation; pancreatic atrophy; restricted diffusion; vascular involvement by the tumor; extrapancreatic tumor spread; and synchronous liver metastases...
May 8, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28479381/integrated-clinicopathological-features-and-gene-microarray-analysis-of-pancreatic-neuroendocrine-tumors
#12
Huaqiang Zhou, Qinchang Chen, Wulin Tan, Zeting Qiu, Si Li, Yiyan Song, Shaowei Gao
Pancreatic neuroendocrine tumors are relatively rare pancreatic neoplasms over the world. Investigations about molecular biology of PNETs are insufficient for nowadays. We aimed to explore the expression of messenger RNA and regulatory processes underlying pancreatic neuroendocrine tumors from different views. The expression profile of GSE73338 were downloaded, including samples with pancreatic neuroendocrine tumors. First, the Limma package was utilized to distinguish the differentially expressed messenger RNA...
May 4, 2017: Gene
https://www.readbyqxmd.com/read/28477012/minimally-invasive-distal-pancreatectomy-for-pnets-laparoscopic-or-robotic-approach
#13
Jiaqiang Zhang, Jiabin Jin, Shi Chen, Jiangning Gu, Yi Zhu, Kai Qin, Qian Zhan, Dongfeng Cheng, Hao Chen, Xiaxing Deng, Baiyong Shen, Chenghong Peng
BACKGROUND: The most effective and radical treatment for pancreatic neuroendocrine tumors (PNETs) is surgical resection. Minimally invasive surgery has been increasingly used in pancreatectomy. Initial results in robotic distal pancreatectomy (RDP) have been encouraging. Nonetheless, data comparing outcomes of RDP with those of laparoscopic distal pancreatectomy (LDP) in treating PNETs are rare. The aim of this study was to compare the safety and efficacy of RDP and LDP for PNETs. METHODS: From September 2010 to January 2017, operative parameters and perioperative outcomes in an initial experience with 43 consecutive patients undergoing RDP were collected and compared with those in 31 patients undergoing LDP...
May 16, 2017: Oncotarget
https://www.readbyqxmd.com/read/28472972/primary-ewing-s-sarcoma-primitive-neuroectodermal-tumor-of-the-ileum-case-report-of-a-16-year-old-chinese-female-and-literature-review
#14
Teng Li, Fang Zhang, Yarui Cao, Shoubin Ning, Yongmin Bi, Weicheng Xue, Li Ren
BACKGROUND: Ewing's sarcoma (ES) and primitive neuroectodermal tumors (PNET) are closely related tumors. Although soft tissue ES/PNET are common in clinical practice, they are rare in the small intestine. Because of the absence of characteristic clinical symptoms, they are easily misdiagnosed as other benign or malignant diseases. CASE PRESENTATION: Here, we present the case of a 16-year-old female who complained of anemia and interval hematochezia. Her serum test results showed only a slight elevation of CA-125 and a low level of hemoglobin...
May 4, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28469346/primitive-neuroectodermal-tumor-of-lung-in-adult-with-hemorrhagic-brain-metastasis-an-extremely-rare-case-scenario
#15
Abhishek Purkayastha, Neelam Sharma, Amul Kapur, Kavita Sahai
Primitive neuroectodermal tumors (PNETs) are highly malignant neoplasms of embryonal origin manifesting in children and adolescents, rarely seen in adults. Carcinoma lung with hemorrhagic metastasis to the brain is very common, but primary lung PNET with hemorrhagic brain metastasis is extremely uncommon. We hereby report a 29-year-old female diagnosed as PNET lung was treated with vincristine, adriamycin, and cyclophosphamide alternating with ifosfamide plus etoposide followed by radiotherapy (RT). After 9 months, she developed hemorrhagic brain metastasis from PNET lung confirmed from tissue immunohistology postcraniotomy...
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28464233/incidence-and-prognostic-value-of-serotonin-secretion-in-pancreatic-neuroendocrine-tumours
#16
Wouter T Zandee, Roxanne C S van Adrichem, Kimberly Kamp, Richard A Feelders, Marie-Louise F van Velthuysen, Wouter W de Herder
BACKGROUND: Serotonin secretion occurs in approximately 1-4% of patients with a pancreatic neuroendocrine tumour (PNET), but the incidence is not well defined. The aim of this study is to determine the incidence of serotonin secretion with and without carcinoid syndrome, and the prognostic value for overall survival (OS). METHODS: Data were collected from 255 patients with a PNET if 24-hour urinary 5-hydroxyindoleacitic acid excretion (5-HIAA) was assessed. Patients were diagnosed with serotonin secretion if 24-hour urinary 5-HIAA excretion was more than 3x the upper limit of normal (ULN) of 50 μmol/24 hours during follow-up...
May 2, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/28462848/outcome-of-resectable-pediatric-ewing-sarcoma-of-the-ribs
#17
Gehad Ahmed, Manal Zamzam, Mohamed S Zaghloul, Ahmed Kamel, Ranin Soliman, Iman Zaky, Asmaa Salama, Nehal Kamal, Maged ElShafiey
PURPOSE: Was to evaluate the outcome of multimodality treatment in resectable primary Ewing sarcoma/primitive neuroectodermal tumor ES/PNET of the ribs and role of thoracoscopy in facilitating resection of these tumors. PATIENTS AND METHODS: This was a retrospective study including 22 patients with primary ES/PNET of the ribs surgically treated at Children's Cancer Hospital Egypt (CCHE) between January 2008 until the end of December 2014. RESULTS: Median age was 8...
April 24, 2017: Journal of the Egyptian National Cancer Institute
https://www.readbyqxmd.com/read/28454419/neutrophil-lymphocyte-ratio-predicts-survival-in-pancreatic-neuroendocrine-tumors
#18
Guopei Luo, Chen Liu, He Cheng, Kaizhou Jin, Meng Guo, Yu Lu, Jiang Long, Jin Xu, Quanxing Ni, Jie Chen, Xianjun Yu
Although the prognostic role of neutrophil-lymphocyte ratio (NLR) has been confirmed in a variety of tumors, the prognostic role of NLR in pancreatic neuroendocrine tumors (PNETs) has not been examined. The present study was performed to assess the role of NLR as a prognostic factor in patients with PNETs. Clinical data were retrospectively retrieved from a single institution. The best cut-off value for baseline NLR levels was determined by the receiver operating characteristic (ROC) curve and area under the ROC curve...
April 2017: Oncology Letters
https://www.readbyqxmd.com/read/28454228/prognostic-factors-of-long-term-outcome-in-surgically-resectable-pancreatic-neuroendocrine-tumors-a-12-year-experience-from-a-single-center
#19
Bo Zhou, Jixuan Duan, Sheng Yan, Jie Zhou, Shusen Zheng
The present study was performed to assess the prognostic factors with respect to patient survival and clinicopathological characteristics for patients who underwent surgery for pancreatic neuroendocrine tumors (PNETs). A retrospective review of patients pathologically diagnosed with PNETs at the First Affiliated Hospital, Zhejiang University School of Medicine (Hangzhou, Zhejiang, China) between September 2002 and September 2013 was conducted. The clinicopathological findings, overall survival (OS) rate and disease-free survival (DFS) rate of the patients with PNETs were analyzed...
March 2017: Oncology Letters
https://www.readbyqxmd.com/read/28454119/anti-proliferative-and-anti-secretory-effects-of-everolimus-on-human-pancreatic-neuroendocrine-tumors-primary-cultures-is-there-any-benefit-from-combination-with-somatostatin-analogs
#20
Amira Mohamed, David Romano, Alexandru Saveanu, Catherine Roche, Manuela Albertelli, Federica Barbieri, Thierry Brue, Patricia Niccoli, Jean-Robert Delpero, Stephane Garcia, Diego Ferone, Tullio Florio, Vincent Moutardier, Flora Poizat, Anne Barlier, Corinne Gerard
Therapeutic management of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) is challenging. The mammalian target of rapamycin (mTOR) inhibitor everolimus recently obtained approval from the Food and Drug Administration for the treatment of patients with advanced pancreatic neuroendocrine tumors (pNETs). Despite its promising antitumor efficacy observed in cell lines, clinical benefit for patients is unsatisfactory. The limited therapeutic potential of everolimus in cancer cells has been attributed to Akt activation due to feedback loops relief following mTOR inhibition...
April 10, 2017: Oncotarget
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