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https://www.readbyqxmd.com/read/28443239/primitive-neuro-ectodermal-tumor-arising-in-a-testicular-teratoma-with-retroperitoneal-metastasis-a-case-report-and-review-of-literature
#1
Guodong Hu, Andrew Wang, Xiu Wang, Leilei Xia, Benjamin L Taylor, S Buce Malkowicz, Priti Lal, Julia R Maisel
A 38-year-old man presenting with left testicular mass and extensive retroperitoneal lymphadenopathy underwent radical orchiectomy and specimen showed a germ cell tumor of primarily primitive neuroectodermal tumor mixed with mature teratoma. He then underwent RPLND, followed by adjuvant CAV (cyclophosphamide, doxorubicin, vincristine) and IE (ifosfamide, etoposide) alternating chemotherapy given the high rate of recurrence and high rate of response to the PNET-specific chemotherapy.
July 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28430639/should-surgery-be-conducted-for-small-nonfunctioning-pancreatic-neuroendocrine-tumors-a-systemic-review
#2
REVIEW
Jingfei Guo, Jianjun Zhao, Xinyu Bi, Zhiyu Li, Zhen Huang, Yefan Zhang, Jianqiang Cai, Hong Zhao
BACKGROUND: The incidence of nonfunctioning pancreatic neuroendocrine tumors smaller than 2cm has increased remarkably in the last two decades. Controversies exist regarding whether surgery should be conducted for this group of tumors. METHODS: MEDLINE, EMBASE and CENTRAL were search until 2017/01/17. Studies with comparative results between operation and observation group were included. Primary outcomes were overall survival and disease specific survival. Secondary outcomes were disease progression and surgical death and complications...
February 25, 2017: Oncotarget
https://www.readbyqxmd.com/read/28416359/marital-status-is-an-independent-prognostic-factor-for-pancreatic-neuroendocrine-tumors-patients-an-analysis-of-the-surveillance-epidemiology-and-end-results-seer-database
#3
Huaqiang Zhou, Yuanzhe Zhang, Yiyan Song, Wulin Tan, Zeting Qiu, Si Li, Qinchang Chen, Shaowei Gao
BACKGROUND AND OBJECTIVES: Marital status's prognostic impact on pancreatic neuroendocrine tumors (PNET) has not been rigorously studied. We aimed to explore the relationship between marital status and outcomes of PNET. METHODS: We retrospectively investigated 2060 PNET cases between 2004 and 2010 from Surveillance, Epidemiology, and End Results (SEER) database. Variables were compared by Chi(2) test, t-test as appropriate. Kaplan-Meier methods and COX proportional hazard models were used to ascertain independent prognostic factors...
April 14, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28405826/everolimus-as-first-line-therapy-for-pancreatic-neuroendocrine-tumours-current-knowledge-and-future-perspectives
#4
REVIEW
Marco Gallo, Pasqualino Malandrino, Giuseppe Fanciulli, Francesca Rota, Antongiulio Faggiano, Annamaria Colao
PURPOSE: Everolimus has been shown to be effective for advanced pancreatic neuroendocrine tumours (pNETs), but its positioning in the therapeutic algorithm for pNETs is matter of debate. METHODS: With the aim to shed light on this point, we performed an up-to-date critical review taking into account the results of both retrospective and prospective published studies, and the recommendations of international guidelines. In addition, we performed an extensive search on the Clinical Trial Registries databases worldwide, to gather information on the ongoing clinical trials related to this specific topic...
April 12, 2017: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/28405084/primitive-neuroectodermal-tumors-of-the-kidney
#5
Geetha Narayanan, Varun Rajan, T R Preethi
Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) rarely occurs as a primary renal tumor. The disease affects young adults and children and has an aggressive course. The clinical presentation and imaging of these tumors are nonspecific, and they often present at an advanced stage. We present the clinical features, imaging, diagnosis, and treatment of 7 cases of renal PNET (4 men, 3 women; median age, 32 years). Common presenting symptoms were flank or abdominal pain and a mass in the abdomen. On imaging, a large heterogenous infiltrating renal mass with areas of calcification, hemorrhage, and necrosis and tumor thrombus can give a clue to the diagnosis of renal PNET...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28404866/combined-antiangiogenic-and-anti-pd-l1-therapy-stimulates-tumor-immunity-through-hev-formation
#6
Elizabeth Allen, Arnaud Jabouille, Lee B Rivera, Inge Lodewijckx, Rindert Missiaen, Veronica Steri, Kevin Feyen, Jaime Tawney, Douglas Hanahan, Iacovos P Michael, Gabriele Bergers
Inhibitors of VEGF (vascular endothelial growth factor)/VEGFR2 (vascular endothelial growth factor receptor 2) are commonly used in the clinic, but their beneficial effects are only observed in a subset of patients and limited by induction of diverse relapse mechanisms. We describe the up-regulation of an adaptive immunosuppressive pathway during antiangiogenic therapy, by which PD-L1 (programmed cell death ligand 1), the ligand of the negative immune checkpoint regulator PD-1 (programmed cell death protein 1), is enhanced by interferon-γ-expressing T cells in distinct intratumoral cell types in refractory pancreatic, breast, and brain tumor mouse models...
April 12, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28393314/real-world-treatment-patterns-in-advanced-pancreatic-neuroendocrine-tumors-in-the-era-of-targeted-therapy-perspectives-from-an-academic-tertiary-center-and-community-oncology-practices
#7
Maurice Herring, Lynn Huynh, Mei Sheng Duh, Francis Vekeman, Audrey Tiew, Maureen Neary, Emily Bergsland
Pancreatic neuroendocrine tumors (pNETs) are rare, slow-growing cancers. Optimal treatment of advanced pNETs is unclear. The aim of this study was to examine treatment patterns and preferences among an academic tertiary medical center and community-based oncology practices. Retrospective chart review was performed for patients with newly diagnosed locally advanced, metastatic, or unresectable pNET diagnosed between January 2010 and December 2013 at an academic tertiary cancer center [University of California, San Francisco (UCSF)] or a large network of community oncology practices [Altos Solutions' OncoEMR database (ALTOS)]...
May 2017: Medical Oncology
https://www.readbyqxmd.com/read/28384877/renal-primitive-neuroectodermal-tumour-case-report-of-a-rare-entity
#8
Sai Chandana Gali, B N Kumarguru, Balachandra Bhat, A S Ramaswamy, M Udaya Kumar
The peripheral Primitive Neuroectodermal Tumour (PNET) is a member of the family of small round cell tumours. PNET is more aggressive in kidney when compared to the other sites. It usually presents in childhood or adolescence. It has an aggressive clinical course and may process towards metastatic disease culminating in death. A 24-year-old female presented with left sided abdominal swelling. Abdominal ultrasound confirmed a heterogeneous left renal mass. Consequently the patient underwent nephrectomy of left kidney and left oophorectomy...
February 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28383090/lumbosacral-epidural-primitive-neuroectodermal-tumor-pnet-case-report-and-literature-review
#9
Keyvan Eghbal, Amir Reza Dehghanian, Fariborz Ghaffarpasand
BACKGROUND: The primitive neuroectodermal tumors (PNET) of the spine are rare and usually are intramedullary and being reported in children. We herein report an epidural PNET of lumbosacral area presenting with cauda equine syndrome in an adult. CASE REPORT: A 38-year-old woman presented to our emergency room with acute onset lower extremity weakness and urinary incontinency. Emergency MRI revealed an isoitense on T1-weighted and heterogeneously hyperintense in T2-weighted images in epidural lumbosacral area...
March 9, 2017: Turkish Neurosurgery
https://www.readbyqxmd.com/read/28378288/development-of-cystic-malacia-after-high-dose-cranial-irradiation-of-pediatric-cns-tumors-in-long-term-follow-up
#10
Fumiyuki Yamasaki, Takeshi Takayasu, Ryo Nosaka, Ikuno Nishibuchi, Hiroshi Kawaguchi, Manish Kolakshyapati, Shumpei Onishi, Taiichi Saito, Kazuhiko Sugiyama, Masao Kobayashi, Kaoru Kurisu
PURPOSE: The purpose of this study is to investigate the incidence of cystic malacia in long-term survivors of pediatric brain tumors treated with high-dose cranial irradiation. MATERIALS AND METHODS: Between 1997 and 2015, we treated 41 pediatric patients (26 males, 15 females; age ranging from 3.3 to 15.7 years, median 9-year-old) of pediatric brain tumors [17 medulloblastomas, 7 primitive neuroectodermal tumors (PNET), 3 pineoblastomas, 6 non-germinomatous germ cell tumors (NGGCT), 8 gliomas (including 4 ependymomas, 1 anaplastic astrocytoma, 1 oligodendroglioma, 1 pilocytic astrocytoma, 1 astroblastoma)] with high-dose craniospinal irradiation...
April 4, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28358174/minerva-chirurgica-2017-mar-27
#11
Eugenia Yiannakopoulou
Minimally invasive surgery has an established role in the treatment of patients with pNETs. Enucleation is an established treatment option for insulinomas. The necessity of organ sparing surgery is well established in the case of multiple pNETs. A number of studies have demonstrated the efficacy and safety of laparoscopic surgery for the treatment of pNETs. However, pNETs are rare, and large studies focusing on pNET patients are difficult to be designed unless multicentre experience is collected. Authors investigating the role of minimally invasive pancreatic surgery should be encouraged to report separately the results that involve patients presenting with pNETs...
March 27, 2017: Minerva Chirurgica
https://www.readbyqxmd.com/read/28357177/pancreatic-neuroendocrine-tumors
#12
REVIEW
Jian Sun
Pancreatic neuroendocrine neoplasms (pNENs) are a heterogeneous group of tumors including well differentiated pancreatic neuroendocrine tumors (pNETs) and neuroendocrine carcinomas (pNECs). The incidence of pNENs has increased over the past few decades. Although, the understanding and interest for this tumor have also increased significantly, the debate about classification and diagnosis continues. Although the primary treatment for pNENs is surgical resection, there is still a lack of effective therapeutic options for patients with advanced unresectable pNENs...
February 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28328619/evaluation-of-o6-methylguanine-dna-methyltransferase-as-a-predicting-factor-of-response-to-temozolomide-based-chemotherapy-in-well-differentiated-metastatic-pancreatic-neuroendocrine-tumors
#13
Paul Girot, Clotilde Dumars, Jean-François Mosnier, Léa Muzellec, Hélène Senellart, Fanny Foubert, François-Xavier Caroli-Bosc, Estelle Cauchin, Nicolas Regenet, Tamara Matysiak-Budnik, Yann Touchefeu
OBJECTIVE: Temozolomide (TMZ) is an alkylating agent frequently used in well-differentiated metastatic pancreatic neuroendocrine tumors (PNETs) with very variable responses. O-methylguanine-DNA methyltransferase (MGMT) is a DNA repair enzyme whose loss of expression has been suggested to be predictive of response to TMZ in various human tumors. We evaluated the predictive value of MGMT status, assessed by immunohistochemistry (IHC) and methylation-specific PCR (MS-PCR), in well-differentiated metastatic PNETs treated by a TMZ-based chemotherapy...
March 21, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28327907/a-randomized-open-label-phase-2-study-of-everolimus-in-combination-with-pasireotide-lar-or-everolimus-alone-in-advanced-well-differentiated-progressive-pancreatic-neuroendocrine-tumors-cooperate-2-trial
#14
M H Kulke, P Ruszniewski, E Van Cutsem, C Lombard-Bohas, J W Valle, W W De Herder, M Pavel, E Degtyarev, J C Brase, L Bubuteishvili-Pacaud, M Voi, R Salazar, I Borbath, N Fazio, D Smith, J Capdevila, R P Riechelmann, J C Yao
Background: Several studies have demonstrated the antitumor activity of first-generation somatostatin analogs (SSAs), primarily targeting somatostatin receptor (sstr) subtypes 2 and 5, in neuroendocrine tumors (NET). Pasireotide, a second-generation SSA, targets multiple sstr subtypes. We compared the efficacy and safety of pasireotide plus everolimus to everolimus alone in patients with advanced, well-differentiated, progressive pancreatic NET (pNET). Patients and methods: Patients were randomized 1:1 to receive a combination of everolimus (10 mg/d, orally) and pasireotide long-acting release (LAR; 60 mg/28 d, intramuscularly) or everolimus alone (10 mg/d, orally); stratified by prior SSA use, and baseline serum chromogranin A and neuron-specific enolase...
March 6, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28325133/outcome-for-children-treated-for-medulloblastoma-and-supratentorial-primitive-neuroectodermal-tumor-cns-pnet-a-retrospective-analysis-spanning-40-years-of-treatment
#15
Einar Stensvold, Bård Kronen Krossnes, Tryggve Lundar, Bernt J Due-Tønnessen, Radek Frič, Paulina Due-Tønnessen, Anne Grete Bechensteen, Tor Åge Myklebust, Tom Børge Johannesen, Petter Brandal
BACKGROUND: Medulloblastoma (MB) and supratentorial primitive neuroectodermal tumor of the central nervous system (CNS-PNET) are among the most common pediatric brain tumors. The diagnosis, treatment, and outcome of MB/CNS-PNET patients treated during the last four decades at Oslo University Hospital (OUH) are described. MATERIAL AND METHODS: All patients younger than 20 years of age diagnosed and treated for MB/CNS-PNET at OUH between 1 January 1974 and 31 December 2013 were identified...
March 21, 2017: Acta Oncologica
https://www.readbyqxmd.com/read/28324047/functional-imaging-in-the-follow-up-of-enteropancreatic-neuroendocrine-tumors-clinical-usefulness-and-indications
#16
Elettra Merola, Marianne E Pavel, Francesco Panzuto, Gabriele Capurso, Noemi Cicchese, Anja Rinke, Thomas M Gress, Elsa Iannicelli, Daniela Prosperi, Patrizia Pizzichini, Vikas Prasad, Patrizia Kump, Rainer Lipp, Stefano Partelli, Massimo Falconi, Bertram Wiedenmann, Gianfranco Delle Fave
Context: Functional imaging tests (FITs) detecting somatostatin receptors expression (i.e., Somatostatin Receptor Scintigraphy, 68Ga-DOTA-peptide positron emission tomography/computed tomography) have a pivotal role in the diagnosis of neuroendocrine tumors (NETs), while their indication during follow-up still needs to be clarified. Objective: Investigate the role of FITs after diagnosis of metastatic enteropancreatic NETs, identifying patients who might benefit from these exams...
January 13, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28323985/utility-of-fdg-pet-imaging-for-risk-stratification-of-pancreatic-neuroendocrine-tumours-in-men-1
#17
E R Kornaczewski Jackson, O P Pointon, R Bohmer, J R Burgess
Context: Patients with Multiple Endocrine Neoplasia Type 1 (MEN 1) are at high risk of malignant pancreatic Neuroendocrine Tumours (pNETs). Structural imaging is typically used to screen for pNETs, but is suboptimal for stratifying malignant potential. Objective: To determine the utility of Fluorodeoxyglucose (18F) positron emission tomography/computed tomography (18F-FDG PET/CT) for predicting the malignant potential of pNETs in MEN 1. Design: Retrospective observational study...
March 7, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28319177/radiological-features-of-primitive-neuroectodermal-tumors-in-intra-abdominal-and-retroperitoneal-regions-a-series-of-18-cases
#18
Xiaoping Yi, Wenguang Liu, Youming Zhang, Desheng Xiao, Hongling Yin, Xueying Long, Li Li, Hongyan Zai, Minfeng Chen, Wenzheng Li, Lunquan Sun
OBJECTIVES: To characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions. METHODS: Eighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed...
2017: PloS One
https://www.readbyqxmd.com/read/28315498/primitive-neuroectodermal-tumour-of-kidney-with-thrombosis-of-the-inferior-vena-cava-and-good-responsive-to-surgical-and-medical-treatment-description-of-a-case-and-revision-of-literature
#19
Giovanni L Giliberto, Carmelo A Di Franco, Bruno Rovereto
Primitive neuroectodermal tumour (PNET) of kidney is a rare cancer typical of young adults with few cases described in the literature. We report a case of renal PNET in a 31-year-old man who presented to our department with a computed tomographic (CT)-scan revealing a large renal mass of 20 cm, massive thrombosis of the inferior vena cava (IVC). The patient underwent radical nephrectomy with contextual retroperitoneal lymphadenectomy and resection of IVC needing Dacron prosthesis substitution. Definitive histopathological examination showed PNET of kidney infiltrating ipsilateral adrenal gland, massive cava thrombosis with infiltration of vena cava wall and one lymph nodal metastasis...
March 15, 2017: Urologia
https://www.readbyqxmd.com/read/28298140/a-cd99-primary-spinal-central-pnet-in-a-pregnant-patient-therapeutic-concerns-and-issues
#20
Aayesha Jalaluddin Soni, Girish Modi, Zakiyya Soni, Jeffrey Kotzen, Zaheera Soni, Vinod Goolab
Spinal tumours in pregnancy are rare. Spinal tumours account for only 10-15% of all primary central nervous system (CNS) tumours. Most spinal tumours in pregnant women have been described as meningiomas or vascular tumours. We present the unique case of a pregnant patient presenting with a CD 99+ primary spinal central PNET.
March 15, 2017: British Journal of Neurosurgery
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