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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/28444219/dna-methylation-of-a-novel-pak4-locus-influences-ototoxicity-susceptibility-following-cisplatin-and-radiation-therapy-for-pediatric-embryonal-tumors
#1
Austin L Brown, Kayla L Foster, Philip J Lupo, Erin C Peckham-Gregory, Jeffrey C Murray, M Fatih Okcu, Ching C Lau, Surya P Rednam, Murali Chintagumpala, Michael E Scheurer
Background: Ototoxicity is a common adverse side effect of platinum chemotherapy and cranial radiation therapy; however, individual susceptibility is highly variable. Therefore, the objective of this study was to conduct an epigenome-wide association study to identify differentially methylated CpG sites associated with ototoxicity susceptibility among cisplatin-treated pediatric patients with embryonal tumors. Methods: Samples were collected for a discovery (n=62) and a replication cohort (n=18) of medulloblastoma and primitive neuroectodermal tumor patients...
April 24, 2017: Neuro-oncology
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
#2
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/28438883/ewing-s-sarcoma-family-tumors-in-the-jaws-case-report-immunohistochemical-analysis-and-literature-review
#3
Ana Regina Casaroto, Marcelo Bonifacio DA Silva Sampieri, Cleverson Teixeira Soares, Paulo Sergio DA Silva Santos, Renato Yassutaka Faria Yaedu, José Humberto Damante, Vanessa Soares Lara
Due to the low incidence of the Ewing's Sarcoma (ES) family tumors, the available epidemiology is likely to be unreliable, and at present, there are no standard diagnostic or clinical guidelines outlining their management. This report describes a case of peripheral primitive neuroectodermal tumor (ES/pPNET) which initially mimicked cystic lesions, and describes a comparison between ES and ES/pPNET in the jaws by the World Health Organization classification. This review addressed 63 cases published in the English literature between 1950 and 2016...
May 2017: In Vivo
https://www.readbyqxmd.com/read/28429277/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-kidney-a-clinicopathologic-study-of-23-cases
#4
Paari Murugan, Priya Rao, Pheroze Tamboli, Bogdan Czerniak, Charles C Guo
Primary Ewing sarcoma / primitive neuroectodermal tumor (ES) of the kidney is a rare neoplasm with limited clinicopathologic data. We report 23 such cases with no history of ES elsewhere in the body. The patients included 13 male and 10 female, aged 8-70 years (mean, 31 years). The average tumor size was 11.7 cm (range, 5-20 cm). Microscopic analysis showed predominantly lobular growth (n = 14), with focal papillary (n = 3), alveolar (n = 1), and hemangiopericytoma-like (n = 1) patterns. Several tumors (n = 11) exhibited robust mitotic activity (>10 mitoses/10 high-power fields)...
April 20, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/28426529/diagnostic-utility-of-pax8-pax2-and-ngfr-immunohistochemical-expression-in-pediatric-renal-tumors
#5
Nicoleta C Arva, Jeffrey Bonadio, Elizabeth J Perlman, Mariana M Cajaiba
Pediatric renal tumors (PRT) with small round blue or spindle cell morphology can be diagnostically challenging and only a limited number of immunohistochemical markers have been documented to help in the diagnosis: paired box (Pax) 2 and nerve growth factor receptor (NGFR) positivity have been demonstrated in Wilms tumor (WT) and clear cell sarcoma of the kidney (CCSK), respectively. However, the immunohistochemical expression of these markers in other PRT remains unknown. This study investigated Pax8, Pax2, and NGFR immunophenotype in a large series of PRT...
April 19, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28408259/pediatric-basal-ganglia-region-tumors-clinical-and-radiological-features-correlated-with-histopathological-findings
#6
Wei Fu, Yan Ju, Si Zhang, Chao You
PURPOSE: To summarize the clinical and radiological features of pediatric basal ganglia region tumors (PBGRT) in correlation with their histopathological findings to reduce inappropriate surgery and identify tumors that can benefit from maximal safe resection. METHODS: The records of 35 children with PBGRT treated in our hospital from December 2011 to December 2015 were retrospectively analyzed. The clinical and radiological features of these tumors were summarized in correlated with their histopathological diagnosis...
April 10, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28405084/primitive-neuroectodermal-tumors-of-the-kidney
#7
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/28403132/orbital-reconstruction-in-neuroectodermal-tumor-of-the-orbit-multimodal-treatment-approach
#8
Jorge Ernesto Cantini A, Diana M Díaz López, Esteban Francisco Hernandez Florez
INTRODUCTION: Primitive neuroectodermal tumors of peripheral origin are very rare, and orbital neuroectodermal tumors are even more uncommon. Only 25 patients with primary orbital involvement in the pediatric age group have been reported. METHODS: In this article, the authors describe their experience in the multimodality treatment approach to treat neuroectodermal tumor of the orbit. The authors also present a male patient 3-year old presenting with a neuroectodermal tumor of the right orbit causing rapidly progressive proptosis...
April 11, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28384657/metronomic-chemotherapy-vs-best-supportive-care-in-progressive-pediatric-solid-malignant-tumors-a-randomized-clinical-trial
#9
Raja Pramanik, Sandeep Agarwala, Yogendra Kumar Gupta, Sanjay Thulkar, Sreenivas Vishnubhatla, Atul Batra, Deepa Dhawan, Sameer Bakhshi
Importance: Although oral metronomic chemotherapy is often used in progressive pediatric solid malignant tumors, a literature review reveals that only small single-arm retrospective or phase 1 and 2 studies have been performed. Skepticism abounds because of the lack of level 1 evidence. Objectives: To compare the effect of metronomic chemotherapy on progression-free survival (PFS) with that of placebo in pediatric patients with primary extracranial, nonhematopoietic solid malignant tumors that progress after at least 2 lines of chemotherapy...
April 6, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28383090/lumbosacral-epidural-primitive-neuroectodermal-tumor-pnet-case-report-and-literature-review
#10
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
#11
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/28375941/two-tumors-in-1-what-should-be-the-therapeutic-target-pediatric-germ-cell-tumor-with-somatic-malignant-transformation
#12
Cecile Faure Conter, Brice Fresneau, Estelle Thebaud, Amandine Bertrand, Frederique Dijoud, Angelique Rome, Cecile Dumesnil, Marie Pierre Castex, Anguella Ghanem, Daniel Orbach
BACKGROUND: Germ cell tumors with somatic malignant transformation (GCT with SMT) are rare in children and poorly described. Data are missing to determine if therapies should target the GCT, the SMT compound, or both simultaneously. PATIENTS AND METHODS: A retrospective national study was conducted in the Société Française des cancers de l'Enfant (SFCE) Centers. Medical records from patients aged 0 to 18 years diagnosed with GCT with SMT between 2000 and 2015 were analyzed...
April 3, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28357093/triple-phase-99m-tc-3p-rgd2-imaging-of-peripheral-primitive-neuroectodermal-tumor-in-the-hip-muscle-group-with-bone-metastasis
#13
Jingjing Fu, Jinhua Song, Youcai Zhao, Feng Wang, Guoqiang Shao
Peripheral primitive neuroectodermal tumors (pPNETs) are a group of aggressive neoplasms that are most commonly encountered in pediatric patients and may be located in the abdomen, pelvis, thoracopulmonary region and, rarely, in the head and neck region. pPNETs in adults are extremely rare. The present study reports a case of pPNET located in the hip muscles with bone metastasis. The patient was a 44-year-old woman who complained of progressive pain and swelling with a mass near the left hip. Computed tomography (CT) and enhanced CT revealed a soft tissue mass lesion in the hip muscle group measuring 4...
February 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28340171/immunohistochemical-approach-to-the-differential-diagnosis-of-meningiomas-and-their-mimics
#14
Camille Boulagnon-Rombi, Clémence Fleury, Caroline Fichel, Sophie Lefour, Aude Marchal Bressenot, Guillaume Gauchotte
The differential diagnosis between meningioma and others tumors can be challenging. This study aimed to evaluate different immunohistochemical markers for the differential diagnosis between meningioma and their morphological mimics. Immunohistochemistry was performed on tissue microarray with antiepithelial membrane antigen (EMA), progesterone receptor, somatostatin receptor 2A (SSTR2A), CD34, STAT6, S100, SOX10, HMB45, MelanA, GFAP, inhibin, and BCL2 antibodies. One hundred and twenty-seven meningiomas, 26 solitary fibrous tumor/hemangiopericytomas (SFT/HPC), 39 schwannomas, 17 hemangioblastomas, 21 melanomas, 9 gliosarcomas, 5 neurofibromas, 9 peripheral primitive neuroectodermal tumors, 7 synovial sarcomas, and 5 malignant peripheral nerve sheath tumors were included in the microarray...
April 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28327927/pineoblastoma-the-experience-at-st-jude-children-s-research-hospital
#15
Kara A Parikh, Garrett T Venable, Brent A Orr, Asim F Choudhri, Frederick A Boop, Amar J Gajjar, Paul Klimo
BACKGROUND: Pineoblastomas are rare, supratentorial, primitive neuroectodermal tumors. OBJECTIVE: To document outcomes with multimodal therapy and evaluate the impact that the degree of surgical resection has on outcome. METHODS: A departmental brain tumor database was queried to identify all patients with pathologically proven pineoblastoma who were treated from January 1997 to June 2015 at St. Jude Children's Research Hospital. For each patient, we recorded demographic, pathological, radiological, surgical, and clinical follow-up data...
March 15, 2017: Neurosurgery
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
#16
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/28319177/radiological-features-of-primitive-neuroectodermal-tumors-in-intra-abdominal-and-retroperitoneal-regions-a-series-of-18-cases
#17
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/28298418/disrupting-the-cd47-sirp%C3%AE-anti-phagocytic-axis-by-a-humanized-anti-cd47-antibody-is-an-efficacious-treatment-for-malignant-pediatric-brain-tumors
#18
Sharareh Gholamin, Siddhartha S Mitra, Abdullah H Feroze, Jie Liu, Suzana A Kahn, Michael Zhang, Rogelio Esparza, Chase Richard, Vijay Ramaswamy, Marc Remke, Anne K Volkmer, Stephen Willingham, Anitha Ponnuswami, Aaron McCarty, Patricia Lovelace, Theresa A Storm, Simone Schubert, Gregor Hutter, Cyndhavi Narayanan, Pauline Chu, Eric H Raabe, Griffith Harsh, Michael D Taylor, Michelle Monje, Yoon-Jae Cho, Ravi Majeti, Jens P Volkmer, Paul G Fisher, Gerald Grant, Gary K Steinberg, Hannes Vogel, Michael Edwards, Irving L Weissman, Samuel H Cheshier
Morbidity and mortality associated with pediatric malignant primary brain tumors remain high in the absence of effective therapies. Macrophage-mediated phagocytosis of tumor cells via blockade of the anti-phagocytic CD47-SIRPα interaction using anti-CD47 antibodies has shown promise in preclinical xenografts of various human malignancies. We demonstrate the effect of a humanized anti-CD47 antibody, Hu5F9-G4, on five aggressive and etiologically distinct pediatric brain tumors: group 3 medulloblastoma (primary and metastatic), atypical teratoid rhabdoid tumor, primitive neuroectodermal tumor, pediatric glioblastoma, and diffuse intrinsic pontine glioma...
March 15, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28296680/primitive-neuroectodermal-tumors-of-the-female-genital-tract-a-morphologic-immunohistochemical-and-molecular-study-of-19-cases
#19
Sarah Chiang, Matija Snuderl, Sakiko Kojiro-Sanada, Ariadna Quer Pi-Sunyer, Dean Daya, Tohru Hayashi, Luisanna Bosincu, Fumihiro Ogawa, Andrew E Rosenberg, Lars-Christian Horn, Lu Wang, A John Iafrate, Esther Oliva
Primary primitive neuroectodermal tumor (PNET) of the female genital tract is rare, and its proper classification remains unclear. The clinical, histologic, and immunophenotypic features as well as EWSR1 rearrangement status of 19 gynecologic PNETs, including 10 ovarian, 8 uterine, and 1 vulvar tumors, are herein reported. Patient age ranged from 12 to 68 years, with a median age of 20 and 51 years among those with ovarian and uterine PNETs, respectively. Morphologic features of central nervous system (CNS) tumors were seen in 15 PNETs, including 9 medulloblastomas, 3 ependymomas, 2 medulloepitheliomas, and 1 glioblastoma, consistent with central PNET...
March 14, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28272661/primary-paediatric-renal-primitive-neuroectodermal-tumor-a-case-report-and-review-of-the-literature
#20
Binny Khandakar, Moumita Maiti, Soumit Dey, Prasenjit Sen Ray, Palas Bhattac Haryya, Ranu Sarkar
Ewing sarcoma/primitive neuroectodermal tumor (PNET) is a high-grade malignant neoplasm commonly affecting bones of the thoracic region. Primary Ewing sarcoma/PNET of the kidney is exceptional; it commonly affects young adults and is rarely reported in children. Here we describe a case of renal PNET in a 10-year-old girl who presented at our institute with haematuria and abdominal lump. Computer tomography scan revealed a huge mass in the right kidney. Computer tomography-guided aspiration from the mass showed cytomorphological features of a small blue round cell tumor...
February 4, 2017: Türk Patoloji Dergisi
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