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https://www.readbyqxmd.com/read/29045981/renal-ewing-s-sarcoma-primitive-neuroectodermal-tumor-a-case-report-and-literature-review
#1
C Liu, L G Cui, H L Wang
Ewing's sarcoma/primitive neuroectodermal tumor (EWS/PNET) in the kidney is a rare but high-grade malignant tumor that affects predominantly elder children and adolescents. Patients mostly present with nonspecific symptoms such as abdominal pain and gross hematuria. Since EWS/PNET has a rapid clinical progression with early metastasis and death, it is essential to make an accurate and early diagnosis. Once diagnosed, multimodality treatment, including radical surgery combined with adjuvant chemotherapy, and radiotherapy if necessary, is recommended...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29042631/prognostic-significance-of-preoperative-gamma-glutamyltransferase-to-lymphocyte-ratio-index-in-nonfunctional-pancreatic-neuroendocrine-tumors-after-curative-resection
#2
Bo Zhou, Canyang Zhan, Jingjing Wu, Jianhua Liu, Jie Zhou, Shusen Zheng
Various inflammation-based prognostic scores have been associated with reduced survival in patients with nonfunctional pancreatic neuroendocrine tumor (NF-PNET). However, few studies have illuminated the relationship between the preoperative gamma-glutamyltransferase (GGT) to lymphocyte ratio index (GLRI) and the prognosis of NF-PNET. A retrospective review of 125 NF-PNET patients following curative resection was conducted. The cut-off values for the inflammation-based prognostic scores, including GLRI, were selected using receiver operating characteristic curve analysis...
October 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29034595/adamantinoma-like-ewing-sarcoma-of-the-parotid-gland-cytopathologic-findings-and-differential-diagnosis
#3
Mohammed T Lilo, Justin A Bishop, Matthew T Olson, Syed Z Ali
Adamantinoma-like Ewing sarcoma (AES) is a rare variant of Ewing sarcoma family of tumors (EFTs), primarily affecting bone and soft tissue. AES has mixed features of Ewing sarcoma (ES)/primitive neuroectodermal tumor (PNET) and adamantinoma with a complex immunoprofile and EWSR1 gene rearrangements. Herein, we report a 72-year-old male who presented with left parotid mass, right neck mass and thyroid nodules. Fine needle aspiration of the left parotid mass displayed nests of monotonous epithelioid cells with basaloid features in a background of small round blue cells and lymphocytes...
October 16, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/29022557/two-childhood-pheochromocytoma-cases-due-to-von-hippel-lindau-disease-one-associated-with-pancreatic-neuroendocrine-tumor-a-rare-manifestation
#4
Aydilek Dağdeviren Çakır, Hande Turan, Ayça Aykut, Asude Durmaz, Oya Ercan, Olcay Evliyaoğlu
(VHL) disease is an autosomal dominantly inherited disorder characterized by hemangioblastomas of retina and central nervous system (CNS); renal cysts, clear cell carcinoma; PCC; endolymphatic sac tumors; cystadenomas of the epididymis in males, broad ligament of uterus in females; pancreatic cysts, cystadenomas and neuroendocrine tumors. We here report two cases of VHL disease presented with PCC as the first manifestation. Hemangioblastoma of CNS in the first case and PNET in the second case developed during follow- up and led to the diagnosis of VHL disease...
October 12, 2017: Journal of Clinical Research in Pediatric Endocrinology
https://www.readbyqxmd.com/read/29019897/endocan-expression-is-correlated-with-poor-progression-free-survival-in-patients-with-pancreatic-neuroendocrine-tumors
#5
Liang-Yu Lin, Yi-Chen Yeh, Chia-Huei Chu, Justin G S Won, Yi-Ming Shyr, Yee Chao, Chung-Pin Li, Shin-E Wang, Ming-Huang Chen
Endocan expression has been reported to be associated with aggressive tumor progression and poor outcomes in various cancers, such as breast cancer, renal cell cancer, lung cancer, gastric cancer, and pituitary adenomas. However, the prognostic significance of endocan in neuroendocrine tumors remains unknown. Thus, the aim of this study was to determine the correlation between endocan expression in pancreatic neuroendocrine tumor (PNET) tissues and progression-free survival. This study included 73 patients with confirmed PNETs who were treated in a single tertiary center in north Taiwan between 1992 and 2015...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29018912/a-nationwide-population-based-study-on-the-survival-of-patients-with-pancreatic-neuroendocrine-tumors-in-the-netherlands
#6
C G Genc, H J Klümpen, M G H van Oijen, C H J van Eijck, E J M Nieveen van Dijkum
BACKGROUND: Large population-based studies give insight into the prognosis and treatment outcomes of patients with pancreatic neuroendocrine tumors (pNETs). Therefore, we provide an overview of the treatment and related survival of pNET in the Netherlands. METHODS: Patients diagnosed with pNET between 2008 and 2013 from the Netherlands Cancer Registry were included. Patient, tumors and treatment characteristics were reported. Survival analyses with log-rank testing were performed to compare survival...
October 10, 2017: World Journal of Surgery
https://www.readbyqxmd.com/read/28993028/the-case-for-dna-methylation-based-molecular-profiling-to-improve-diagnostic-accuracy-for-central-nervous-system-embryonal-tumors-not-otherwise-specified-in-adults
#7
Gail C Halliday, Reimar C Junckerstorff, Jacqueline M Bentel, Andrew Miles, David T W Jones, Volker Hovestadt, David Capper, Raelene Endersby, Catherine H Cole, Tom van Hagen, Nicholas G Gottardo
Central nervous system primitive neuro-ectodermal tumors (CNS-PNETs), have recently been re-classified in the most recent 2016 WHO Classification into a standby catch all category, "CNS Embryonal Tumor, not otherwise specified" (CNS embryonal tumor, NOS) based on epigenetic, biologic and histopathologic criteria. CNS embryonal tumors (NOS) are a rare, histologically and molecularly heterogeneous group of tumors that predominantly affect children, and occasionally adults. Diagnosis of this entity continues to be challenging and the ramifications of misdiagnosis of this aggressive class of brain tumors are significant...
October 6, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28988702/prognosis-of-sporadic-resected-small-%C3%A2-2%C3%A2-cm-nonfunctional-pancreatic-neuroendocrine-tumors-a-multi-institutional-study
#8
Ville J Sallinen, Tessa T Y Le Large, Elke Tieftrunk, Shamil Galeev, Zahar Kovalenko, Sven-Petter Haugvik, Anne Antila, Oskar Franklin, Emma Martinez-Moneo, Stuart M Robinson, Francesco Panzuto, Nicolas Regenet, Francesca Muffatti, Stefano Partelli, Dominik Wiese, Philippe Ruszniewski, Bertrand Dousset, Bjørn Edwin, Detlef K Bartsch, Alain Sauvanet, Falconi Massimo, Güralp O Ceyhan, Sebastien Gaujoux
BACKGROUND: Malignant potential of small (≤20 mm) nonfunctional pancreatic neuroendocrine tumors (sNF-PNET) is difficult to predict and management remain controversial. The aim of this study was to assess the prognosis of sporadic nonmetastatic sNF-PNETs. METHODS: Patients were identified from databases of 16 centers. Outcomes and risk factors for recurrence were identified by uni- and multivariate analyses. RESULTS: sNF-PNET was resected in 210 patients, and 66% (n = 138) were asymptomatic...
October 5, 2017: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
https://www.readbyqxmd.com/read/28984786/clinically-significant-differences-in-ki-67-proliferation-index-between-primary-and-metastases-in-resected-pancreatic-neuroendocrine-tumors
#9
Sebastian Richards-Taylor, Charles Tilley, Eleanor Jaynes, Haixiao Hu, Thomas Armstrong, Neil W Pearce, Rachel Plant, Judith Cave
OBJECTIVE: Pancreatic neuroendocrine tumors (NETs) (pNETs) have a varied prognosis according to their grade. The European Neuroendocrine Tumor Society grading system uses assessment of the proliferation index via Ki-67 immunohistochemistry to aid prognosis. There is evidence that the proliferation index can vary significantly within a single tumor, but it is not fully understood to what extent heterogeneity occurs between the primary and metastatic sites and how this may affect the grade...
November 2017: Pancreas
https://www.readbyqxmd.com/read/28977163/use-of-cinacalcet-and-sunitinib-to-treat-hypercalcaemia-due-to-a-pancreatic-neuroendocrine-tumor
#10
Hernan Valdes-Socin, Matilde Rubio Almanza, Mariana Tomé Fernández-Ladreda, Daniel Van Daele, Marc Polus, Marcela Chavez, Albert Beckers
Neuroendocrine tumors (NETs) can secrete hormones, including ectopic secretions, but they have been rarely associated with malignant hypercalcemia. A 52-year-old man with a history of diabetes mellitus was diagnosed with a pancreatic tumor. A pancreatic biopsy confirmed a well-differentiated pancreatic NET (pNET). The patient subsequently developed liver metastasis and hypercalcemia with high 1,25 OH vitamin D and suppressed parathyroid hormone (PTH) levels. Hypercalcemia was refractory to chemotherapy, intravenous saline fluids, diuretics, calcitonin and zoledronate...
September 18, 2017: Archives of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28964660/prediction-of-lymph-node-metastasis-in-pancreatic-neuroendocrine-tumors-by-contrast-enhancement-characteristics
#11
Takuya Mizumoto, Hirochika Toyama, Sachio Terai, Hideyo Mukubou, Hironori Yamashita, Sachiyo Shirakawa, Yoshihide Nanno, Keitaro Sofue, Masahiro Kido, Tetsuo Ajiki, Takumi Fukumoto
BACKGROUND: Iso- or hypo-attenuating areas in the arterial phase on contrast-enhanced computed tomography (CE-CT) have been reported to be negative prognostic features in pancreatic neuroendocrine tumors (PNETs). Given that the optimal indication for lymph node dissection in patients with PNET remains unclear, we sought to utilize enhancement characteristics on CE-CT as a preoperative predictor of regional lymph node metastasis in PNETs. METHODS: The medical records of patients with well-differentiated PNETs who underwent pancreatectomy along with lymphadenectomy were retrospectively analyzed...
August 19, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/28953206/anticancer-effects-of-baicalein-in-pancreatic-neuroendocrine-tumors-in-vitro-and-in-vivo
#12
Ye Tian, Liang Zhen, Jian'an Bai, Yumei Mei, Zheng Li, Aihua Lin, Xueliang Li
OBJECTIVES: Baicalein is a Chinese traditional medicine that inhibits tumor migration and growth. Pancreatic neuroendocrine tumors (pNETs) have a high incidence in China, but there are still no effective treatments. The aim of our study was to investigate whether baicalein could inhibit pNETs. METHODS: In vitro, we used BON1-a cell line of pNETs-to analyze the apoptosis and migration and invasion after baicalein treatment via flow cytometry and Western blot. In vivo, we used a xenograft tumors model to evaluate the size of tumors after baicalein treatment...
August 4, 2017: Pancreas
https://www.readbyqxmd.com/read/28941156/strong-antitumor-efficacy-of-a-pancreatic-tumor-targeting-oncolytic-adenovirus-for-neuroendocrine-tumors
#13
Yuki Yamamoto, Masaki Nagasato, Yosei Rin, Marina Henmi, Yoshinori Ino, Shinichi Yachida, Rieko Ohki, Nobuyoshi Hiraoka, Masatoshi Tagawa, Kazunori Aoki
Although oncolytic adenoviruses are promising cancer therapy agents, for effective oncolytic activity, viruses need to specifically infect and effectively replicate in cancer cells but not in normal cells. We have previously identified a pancreatic cancer-targeting ligand, SYENFSA (SYE), by screening an adenovirus library displaying random peptides against human pancreatic cancer cells and reported that a survivin promoter-regulated adenovirus, displaying the SYE ligand (AdSur-SYE), provided effective oncolysis of pancreatic ductal adenocarcinoma (PDAC) in a preclinical study...
October 2017: Cancer Medicine
https://www.readbyqxmd.com/read/28938468/long-term-natural-course-of-small-non-functional-pancreatic-neuroendocrine-tumors-in-men1-results-from-the-dmsg
#14
Carolina R C Pieterman, Joanne M de Laat, Jos W R Twisk, Rachel S van Leeuwaarde, Wouter W de Herder, Koen M A Dreijerink, Ad R M M Hermus, Olaf M Dekkers, Anouk N A van der Horst-Schrivers, Madeleine L Drent, Peter H Bisschop, Bastiaan Havekes, Inne H M Borel Rinkes, Menno R Vriens, Gerlof D Valk
Background: Pancreatic neuroendocrine tumors (pNETs) are highly prevalent in patients with Multiple Endocrine Neoplasia type 1 (MEN1) and metastatic disease is an important cause of MEN1-related mortality. Especially small non-functional (NF) pNETs pose a challenge to the treating physician and more information is needed regarding their natural course. We assessed long-term natural history of small NF-pNETs and its modifiers in the Dutch MEN1 population. Patients and Methods: Retrospective longitudinal observational cohort study of patients with small (<2cm) NF-pNETs from the Dutch national MEN1 database which includes >90% of the Dutch MEN1 population...
August 4, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28929779/pancreatic-neuroendocrine-tumors-prognostic-factors
#15
Javier A Cienfuegos, Fernando Rotellar, Miguel Ruiz-Canela
We thank Modesto Varas et al. for their opportune comments with regard to the oncologic outcomes of our series of patients undergoing surgery for pancreatic neuroendocrine tumors (PNET). We agree with Varas et al. in relation to the increase in non-functional tumors (73%) and the incidental form of presentation (44%). These figures are in line with those presented in most series.
October 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28924486/expression-of-transcription-factors-in-men1-associated-pancreatic-neuroendocrine-tumors
#16
Yasutaka Takeda, Yukihiro Fujita, Kentaro Sakai, Tomoe Abe, Tomonobu Nakamura, Tsuyoshi Yanagimachi, Hidemitsu Sakagami, Jun Honjo, Atsuko Abiko, Yuichi Makino, Masakazu Haneda
MEN1-associated pancreatic neuroendocrine tumors (pNETs) may potentially express distinct hormones, but the mechanism has not been elucidated. Transcription factors such as MafA and Pdx1 have been identified to lead to beta cell differentiation, while Arx and Brn4 to alpha cell differentiation in developing pancreas. We hypothesized those transcription factors are important to produce specific hormones in pNETs, similarly to developing pancreas, and examined the expression of transcription factors in a case of MEN1 who showed immunohistological coexistence of several hormone-producing pNETs including insulinoma...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28903419/development-of-zebrafish-medulloblastoma-like-pnet-model-by-talen-mediated-somatic-gene-inactivation
#17
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 tp53(e7/e7) mutant zebrafish...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28903383/association-between-abo-blood-types-and-sporadic-pancreatic-neuroendocrine-tumors-in-the-chinese-han-population
#18
Qiwen Ben, Jun Liu, Weiyi Wang, Fang Guo, Weiyan Yao, Jie Zhong, Yaozong Yuan
BACKGROUND: Although the relationship between non-O blood types and the risk of exocrine pancreatic cancer has been demonstrated, the association between ABO blood types and sporadic pancreatic neuroendocrine tumor (PNET) has not been reported thus far. METHODS: This hospital-based, case-control study included 387 patients with PNET and 542 age- and sex-matched controls. Unconditional multivariable logistic regression analysis was performed to estimate the adjusted odds ratios (AORs) and 95% confidence intervals (CIs)...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28890993/chemotherapy-in-nets-when-and-how
#19
REVIEW
Anna Angelousi, Gregory Kaltsas, Anna Koumarianou, Martin O Weickert, Ashley Grossman
The majority of neuroendocrine tumours (NETs) are well-differentiated tumours that follow an indolent course, in contrast to a minority of poorly differentiated neuroendocrine carcinomas (NECs) which exhibit an aggressive course and assocaited with an overall short survival. Although surgery is the only curative treatment for NETs it is not always feasible,necessitating the application of other therapies including chemotherapy. Streptozotocin (STZ)-based regimens have long been used for advanced or metastatic well-to-moderately differentiated (G1-G2) NETs, especially those originating from the pancreas (pNETs)...
September 11, 2017: Reviews in Endocrine & Metabolic Disorders
https://www.readbyqxmd.com/read/28887784/pancreatic-neuroendocrine-tumor-in-a-patient-with-a-tsc1-variant-case-report-and-review-of-the-literature
#20
REVIEW
Parisa Mortaji, Katherine T Morris, Von Samedi, Steven Eberhardt, Shawnia Ryan
The majority of pancreatic neuroendocrine tumors (PNETs) are sporadic while 10-15% are attributable to one of several familial cancer syndromes. Hereditary forms are more commonly associated with Multiple Endocrine Neoplasia Type I and von Hippel Lindau Syndrome. However, patients with Tuberous sclerosis complex also have an increased incidence of PNETs. More often this has been reported in patients with TSC2 variants. In this case report, we summarize the literature regarding PNETs associated with Tuberous sclerosis complex, as well as present a case of a patient with a TSC1 variant and a PNET...
September 8, 2017: Familial Cancer
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