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https://www.readbyqxmd.com/read/29914980/epigenetic-regulators-rbbp4-and-hdac1-are-overexpressed-in-a-zebrafish-model-of-rb1-embryonal-brain-tumor-and-are-required-for-neural-progenitor-survival-and-proliferation
#1
Laura E Schultz, Jeffrey A Haltom, Maira P Almeida, Wesley A Wierson, Staci L Solin, Trevor J Weiss, Jordan A Helmer, Elizabeth J Sandquist, Heather R Shive, Maura McGrail
In this study, we used comparative genomics and developmental genetics to identify epigenetic regulators driving oncogenesis in a zebrafish retinoblastoma 1 ( rb1 ) somatic-targeting model of RB1 mutant embryonal brain tumors. Zebrafish rb1 brain tumors caused by TALEN or CRISPR targeting are histologically similar to human central nervous system primitive neuroectodermal tumors (CNS-PNETs). Like the human oligoneural OLIG2+/SOX10+ CNS-PNET subtype, zebrafish rb1 tumors show elevated expression of neural progenitor transcription factors olig2 , sox10 , sox8b and the receptor tyrosine kinase erbb3a oncogene...
June 15, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29891750/ewing-sarcoma-peripheral-primitive-neuroectodermal-tumor-in-the-adrenal-gland-of-a-child
#2
Kourosh Goudarzipour, Farzaneh Farahmandi, Ahmad Mohammadi, Reza Taherian
Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PNET) typically occurs in long or flat bones, soft tissues, or less often, solid organs. Ewing sarcoma/peripheral primitive neuroectodermal tumor arising from the adrenal gland is extremely rare, especially in children, and only limited cases are reported previously. Herein, we review a case of a 22-month-old girl who presented to our department with abdominal pain, bulging of the left flank, and a nonfunctioning adrenal lesion which was found to be an adrenal ES/PNET...
May 2018: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/29805790/primitive-neuroectodermal-tumor-of-the-kidney-at-the-advanced-stage-a-case-series-of-eight-chinese-patients
#3
Qipeng Sun, Bin Miao, Xiangming Lao, Ping Yuan, Jiarong Cai, Hailun Zhan
Primitive neuroectodermal tumor (PNET) rarely occurs as a primary renal neoplasm. Renal (r)PNET is a rare but aggressive neoplasm with poor prognosis; the majority of patients are diagnosed as advanced stage at presentation and face a worse prognosis than patients with localized disease. The present study describes the diagnosis and management of eight cases of rPNET at an advanced stage, who were treated at two institutions [Lingnan Hospital (branch of The Third Affiliated Hospital) and the Cancer Center of Sun Yat-sen University, Guangzhou], from December 2004 to January 2013...
June 2018: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29805630/low-accuracy-of-chromogranin-a-for-diagnosing-early-stage-pancreatic-neuroendocrine-tumors
#4
Chao-Ming Tseng, Tsu-Yao Cheng, Tai-Been Chen, Yu-Wen Tien, Chien-Chuan Chen, Jaw-Town Lin, Hsiu-Po Wang
The aim of the present study was to evaluate the clinical utility of plasma chromogranin A (CgA) in patients diagnosed with early-stage pancreatic neuroendocrine tumors (PNETs) in terms of diagnostic value and treatment response. A total of 35 patients with PNETs were prospectively enrolled from August 2010 to April 2014. Demographic and clinicopathological data were collected, and serial plasma CgA levels were measured. Tumor responses were defined by the Response Evaluation Criteria In Solid Tumors criteria...
June 2018: Oncology Letters
https://www.readbyqxmd.com/read/29789013/prognostic-significance-of-pd-l1-expression-and-cd8-t-cell-infiltration-in-pulmonary-neuroendocrine-tumors
#5
Haiyue Wang, Zhongwu Li, Bin Dong, Wei Sun, Xin Yang, Ruping Liu, Lixin Zhou, Xiaozheng Huang, Ling Jia, Dongmei Lin
BACKGROUND: Recent research supports a significant role of immune checkpoint inhibitors in the treatment of solid tumors. However, relevant reports for programmed death-ligand 1 (PD-L1) and CD8+ tumor-infiltrating lymphocytes (TILs) in pulmonary neuroendocrine tumors (PNETs) have not been fully studied. Therefore, we investigated PNETs for the expression of PD-L1 and infiltration by CD8+ TILs as well as the prognostic value of both factors. METHODS: In total, 159 specimens of PNETs (35 TC, 2 AC, 28 LCNEC, 94 SCLC) were included in this study...
May 22, 2018: Diagnostic Pathology
https://www.readbyqxmd.com/read/29782352/an-update-on-the-management-of-pancreatic-neuroendocrine-tumors
#6
Limin Gao, Nikola S Natov, Kevin P Daly, Faisal Masud, Sadia Chaudhry, Mark J Sterling, Muhammad W Saif
Pancreatic neuroendocrine tumors (PNETs) are a rare and heterogeneous group of neoplasia and differ in their clinical presentation, behavior, and prognosis based on both histological features and cancer stage at the time of diagnosis. Although small-sized tumors can be surgically resected, locally advanced and metastatic tumors confer a poor prognosis. In addition, only limited treatment options are available to the latter group of patients with PNETs, such as hormonal analogs, cytotoxic agents, and targeted therapy...
May 8, 2018: Anti-cancer Drugs
https://www.readbyqxmd.com/read/29760809/the-latest-exploration-of-staging-and-prognostic-classification-for-pancreatic-neuroendocrine-tumors-a-large-population-based-study
#7
Shanshan Gao, Ning Pu, Lingxiao Liu, Changyu Li, Xuefeng Xu, Xiaolin Wang, Wenhui Lou
Background: A modified European Neuroendocrine Tumor Society (mENETS) staging system has been confirmed to be more suitable for pancreatic neuroendocrine tumors (pNETs) when compared to the American Joint Committee on Cancer (AJCC) or the European Neuroendocrine Tumor Society (ENETS) systems in the last few years. However, the importance of N stage has been recently published with several significant updates. Methods: SEER registry (n = 2,209) was used to evaluate the application of the AJCC 7th staging system, ENETS staging system, mENETS staging system and reformed ENETS (rENETS) staging system in this study...
2018: Journal of Cancer
https://www.readbyqxmd.com/read/29760558/pretreatment-hematologic-markers-as-prognostic-predictors-of-gastroenteropancreatic-neuroendocrine-tumors-a-systematic-review-and-meta-analysis
#8
Yu Zhou, Dezhi Li, Ye Lin, Min Yu, Xin Lu, Zhixiang Jian, Ning Na, Baohua Hou
Background: Systemic inflammation can be reflected by peripheral hematologic parameters and combined index like the lymphocyte count, neutrophil count, platelet count, neutrophil-to-lymphocyte (NLR), and platelet-to-lymphocyte ratio (PLR). This systematic review and meta-analysis aimed to summarize the association between the hematologic markers and prognosis of gastroenteropancreatic neuroendocrine tumors (GEP-NETs). Methods: A computerized systematic search of PubMed, Embase, and Web of Science was conducted up to August 2016...
2018: OncoTargets and Therapy
https://www.readbyqxmd.com/read/29752349/paediatric-pancreatic-neuroendocrine-tumours-in-von-hippel-lindau-disease
#9
Samuel Matthew O'Toole, Anju Sahdev, Satya Bhattacharya, Roger Feakins, Evelien F Gevers, William Drake
Extract: Pancreatic neuroendocrine tumours (pNETs) are an established feature of von Hippel-Lindau disease (VHL), occurring in up to 17% of mutation carriers (Libutti et al. 2000; Blansfield et al. 2007; Erlic et al. 2010; Igarashi et al. 2014). The natural history of VHL-pNETs is poorly characterised with metastatic disease occurring in up to 25% of affected individuals (Erlic et al. 2010). Management of this unique pNET subgroup is complicated by the potential for multifocal and metachronous disease as well as extra-pancreatic VHL-related neoplasms (e...
May 11, 2018: Endocrine-related Cancer
https://www.readbyqxmd.com/read/29725435/uncovering-the-heterogeneous-genetic-variations-in-two-insulin-expressing-tumors-in-a-patient-with-men1
#10
Zai Wang, Liguo Liu, Jie Luo, Jing Guo, Min Zhai, Wenjian Zhang, Zhiying Yang
Multiple endocrine neoplasia type 1 (MEN1) is associated with a heterozygous inherited mutation of the menin 1 ( MEN1 ) gene; however, the molecular pathogenesis remains to be fully elucidated. In the present study, whole exome sequencing was performed on two pancreatic neuroendocrine tumors (PNETs), termed T1 and T2, peri-tumoral tissue (PT) and a blood sample obtained from a patient with MEN1. The cells in T1 and T2, but not PT, showed loss of chromosome 11 where MEN1 was located, confirming that the loss of heterozygosity (LOH) of MEN1 was a crucial event in tumorigenesis...
May 2018: Oncology Letters
https://www.readbyqxmd.com/read/29698969/endoscopic-ultrasound-features-of-multiple-endocrine-neoplasia-type-1-related-versus-sporadic-pancreatic-neuroendocrine-tumors-a-single-center-retrospective-study
#11
Gianluca Tamagno, Vanessa Scherer, Alberto Caimo, Simona R Bergmann, Peter H Kann
AIM: Pancreatic neuroendocrine tumors (pNETs) can occur in patients with a familial syndrome either as multiple endocrine neoplasia type 1 (MEN-1) or as sporadic tumors. Endoscopic ultrasound (EUS) has become one of the first-line investigations for pNET characterization. The ultrasonographic features of pNETs may differ depending on the familial versus sporadic pathogenesis of the tumor. Therefore, the EUS findings could help and direct the definition of a pNET with an impact on the most appropriate diagnostic and therapeutic patient management...
April 26, 2018: Digestion
https://www.readbyqxmd.com/read/29696624/therapeutic-targeting-of-tumor-associated-macrophages-in-pancreatic-neuroendocrine-tumors
#12
Sebastian Krug, Rami Abbassi, Heidi Griesmann, Bence Sipos, Dominik Wiese, Peter Rexin, Annika Blank, Aurel Perren, Johannes Haybaeck, Stefan Hüttelmaier, Anja Rinke, Thomas M Gress, Patrick Michl
Pancreatic neuroendocrine tumors (PNETs) represent a heterogeneous group of neuroendocrine neoplasms with varying biological behaviour and response to treatment. Although targeted therapies have been shown to improve the survival for patients at advanced stage, resistance to current therapies frequently occurs during the course of therapy. Previous reports indicate that the infiltration of tumor-associated macrophages (TAMs) in PNETs might correlate with tumor progression and metastasis formation. We aimed to evaluate the prognostic and functional impact of TAMs in human PNETs in vitro and in vivo and to investigate the effect of therapeutic targeting TAMs in a genetic PNET mouse model...
April 26, 2018: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/29683974/pancreatic-neuroendocrine-tumors-and-intraductal-papillary-mucinous-neoplasm-of-the-pancreas-a-systematic-review
#13
Alba Manuel-Vazquez, José Manuel Ramia, Raquel Latorre-Fragua, Ainhoa Valle-Rubio, Vladimir Arteaga-Peralta, Carmen Ramiro-Pérez, Roberto de la Plaza-Llamas
OBJECTIVES: This study aimed to identify factors that explain the association of intraductal papillary mucinous neoplasms-pancreatic neuroendocrine tumors (IPMNs-PNETs), radiological characteristics, and factors that might guide therapy. METHODS: We performed a systematic review of the literature to search for articles on concurrent IPMN-PNET, mixed endocrine-exocrine pancreatic tumors, and/or PNET with an intraductal growth pattern. RESULTS: A review of the literature suggests that there is some confusion about association of IPMNs-PNETs...
May 2018: Pancreas
https://www.readbyqxmd.com/read/29682023/extraosseous-primary-intracranial-ewing-sarcoma-peripheral-primitive-neuroectodermal-tumor-series-of-seven-cases-and-review-of-literature
#14
Amit Kumar Singh, Arun Kumar Srivastava, Lily Pal, Jayesh Sardhara, Rajan Yadav, Shalini Singh, Kamlesh Singh Bhaisora, Kuntal Kanti Das, Anant Mehrotra, Rabi Narayan Sahu, Awadhesh Kumar Jaiswal, Sanjay Behari
Background: The Ewing sarcoma peripheral PNET (ES-pPNET) is very rare small round cell tumour that involves the CNS as either a primary dural neoplasm or by direct extension from contiguous bone or soft tissue. Materials and Methods: Biopsy proven cases of intracranial ES/pPNET with orbital involvement operated during Jan 2010-Jan 2014 were retrospectively included and their clinical data, operative and histological findings were reviewed from institutional oncology register...
April 2018: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/29676474/a-rare-case-of-ewing-s-sarcoma-primitive-neuroectodermal-tumor-metastasizing-to-left-atrium-through-left-inferior-pulmonary-vein
#15
Suraj K Kulkarni, Nagaraj Moorthy, Shankar Somanna, Manjunath Cholenahally Nanjappa
Cardiac metastasis of Ewing's sarcoma is exceedingly rare. A 40-year-old male was admitted with complaints of nonproductive cough, exertional dyspnea, and fatigue since 4 months with a history of abdominal wall swelling which was excised and proven to be Ewing's sarcoma/primitive neuroectodermal tumor (PNET). A transthoracic echocardiography demonstrated inhomogeneous mass located posterior to the left ventricle and a solitary mass protruding into the left atrium through the left inferior pulmonary vein. Due to accompanying pulmonary metastasis and possible poor outcome of the surgery, surgical resection was not considered...
June 2018: Echocardiography
https://www.readbyqxmd.com/read/29676370/primary-primitive-neuroectodermal-tumor-of-the-kidney
#16
Sunil Vitthalrao Jagtap, Pradnya Pandurang Kale, Anil Huddedar, Atul Bhanudas Hulwan, Swati S Jagtap
Renal primitive neuroectodermal tumor (PNET) is a rare entity. It should be differentiated from other primary renal malignancies. The differentiation of the malignant small round cell tumors may be challenging; however, it is essential for better management of the patient. We report a 45-year-old male having complaints of pain in the abdomen, hematuria, and mass in the abdomen for 6 months. Abdominal and pelvic sonography finding showed cystic-solid, right renal mass suggestive of malignancy. On histopathology diagnosed as malignant small round blue cell tumor suggestive of primitive neuroectodermal tumor...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29676369/a-mesenteric-primary-peripheral-ewing-s-sarcoma-primitive-neuroectodermal-tumor-with-molecular-cytogenetic-analysis-report-of-a-rare-case-and-review-of-literature
#17
Yi-Shu Liao, I-Han Chiang, Hong-Wei Gao
Rare cases of Ewing's sarcoma/primitive neuroectodermal tumors (EWS/PNETs) arising from mesenteric tissue have been reported. This report describes an EWS/PNET in a 25-year-old woman who presented with abdominal pain lasting 3 days. Radiologic evaluation revealed a 9 cm × 6 cm homogeneous mass in the lower abdomen with homogeneous enhancement and invasion of the ileum. Surgical resection was completed during exploratory laparotomy. Immunohistochemically, the tumor cells revealed CD99, friend leukemia virus integration-1 and NKX2...
April 2018: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29655834/metformin-use-associates-with-longer-progression-free-survival-of-patients-with-diabetes-and-pancreatic-neuroendocrine-tumors-receiving-everolimus-and-or-somatostatin-analogues
#18
Sara Pusceddu, Claudio Vernieri, Massimo Di Maio, Riccardo Marconcini, Francesca Spada, Sara Massironi, Toni Ibrahim, Maria Pia Brizzi, Davide Campana, Antongiulio Faggiano, Dario Giuffrida, Maria Rinzivillo, Sara Cingarlini, Francesca Aroldi, Lorenzo Antonuzzo, Rossana Berardi, Laura Catena, Chiara De Divitiis, Paola Ermacora, Vittorio Perfetti, Annalisa Fontana, Paola Razzore, Carlo Carnaghi, Maria Vittoria Davì, Carolina Cauchi, Marilina Duro, Sergio Ricci, Nicola Fazio, Federica Cavalcoli, Alberto Bongiovanni, Anna La Salvia, Nicole Brighi, Annamaria Colao, Ivana Puliafito, Francesco Panzuto, Silvia Ortolani, Alberto Zaniboni, Francesco Di Costanzo, Mariangela Torniai, Emilio Bajetta, Salvatore Tafuto, Silvio Ken Garattini, Daniela Femia, Natalie Prinzi, Laura Concas, Giuseppe Lo Russo, Massimo Milione, Luca Giacomelli, Roberto Buzzoni, Gianfranco Delle Fave, Vincenzo Mazzaferro, Filippo de Braud
BACKGROUND & AIMS: Metformin seems to have anti-cancer effects. However, it is not clear whether use of glycemia and metformin affect outcomes of patients with advanced pancreatic neuroendocrine tumors (pNETs). We investigated the association between glycemia and progression-free survival (PFS) of patients with NETs treated with everolimus and/or somatostatin analogues, as well as the association between metformin use and PFS time. METHODS: We performed a retrospective analysis of 445 patients with advanced pNET treated at 24 medical centers in Italy, from 1999 through 2015...
April 12, 2018: Gastroenterology
https://www.readbyqxmd.com/read/29643685/unilateral-brown-fat-suppression-on-fdg-pet-ct-detecting-sympathetic-denervation
#19
Saurabh Arora, Nishikant Avinash Damle, K Sreenivasa Reddy, Girish Kumar Parida, Abhinav Singhal, Sreedharan Thankarajan Arunraj, Chandrasekhar Bal, Roma Singh, Shobhana Raju, Dhritiman Chakraborty
We present here a case of primitive neuroectodermal tumor (PNET) who initially presented with involvement of the right 3rd rib and underwent neoadjuvant chemotherapy, rib excision, and adjuvant chemoradiotherapy and later underwent posterolateral thoracotomy, pleural nodule excision, and the right 11th rib metastatic lesion excision. Follow-up 18 F-FDG PET/CT/computed tomography revealed unilateral brown fat suppression in the form of decreased metabolic uptake in the ipsilateral cervical, axillary, and paravertebral brown fat as compared to metabolically active contralateral brown fat, likely due to paravertebral sympathetic chain damage...
April 2018: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/29629846/molecular-profiling-of-pancreatic-neuroendocrine-tumors-pnets-and-the-clinical-potential
#20
Massimiliano Camilli, Konstantinos Papadimitriou, Amanda Nogueira, Lorena Incorvaia, Antonio Galvano, Federica D'Antonio, Jose Ferri, Daniele Santini, Nicola Silvestris, Antonio Russo, Marc Peeters, Christian Rolfo
Pancreatic neuroendocrine tumors (pNETs) represent a small part of pancreatic neoplasms, and the knowledge about their indolent clinical course remains a subject of investigation. They occur sporadically or as part of familial cancer syndromes and are classified by WHO in 3 categories. There is ongoing research to understand their molecular profiling and leading mutations. Areas covered: The aim of this review is to clarify the overall aspects of tumorigenesis, to expose the latest developments in understanding the course of the disease and the possible therapeutic implications of these...
May 2018: Expert Review of Gastroenterology & Hepatology
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