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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/28340171/immunohistochemical-approach-to-the-differential-diagnosis-of-meningiomas-and-their-mimics
#1
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...
March 14, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28327927/pineoblastoma-the-experience-at-st-jude-children-s-research-hospital
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
https://www.readbyqxmd.com/read/28249000/stabilization-of-hif-1%C3%AE-and-hif-2%C3%AE-up-regulation-of-mycc-and-accumulation-of-stabilized-p53-constitute-hallmarks-of-cns-pnet-animal-model
#8
Sergey Malchenko, Simone Treiger Sredni, Yingtao Bi, Naira V Margaryan, Jerusha Boyineni, Indra Mohanam, Tadanori Tomita, Ramana V Davuluri, Marcelo B Soares
Recently, we described a new animal model of CNS primitive neuroectodermal tumors (CNS-PNET), which was generated by orthotopic transplantation of human Radial Glial (RG) cells into NOD-SCID mice's brain sub-ventricular zone. In the current study we conducted comprehensive RNA-Seq analyses to gain insights on the mechanisms underlying tumorigenesis in this mouse model of CNS-PNET. Here we show that the RNA-Seq profiles derived from these tumors cluster with those reported for patients' PNETs. Moreover, we found that (i) stabilization of HIF-1α and HIF-2α, which are involved in mediation of the hypoxic responses in the majority of cell types, (ii) up-regulation of MYCC, a key onco-protein whose dysregulation occurs in ~70% of human tumors, and (iii) accumulation of stabilized p53, which is commonly altered in human cancers, constitute hallmarks of our tumor model, and might represent the basis for CNS-PNET tumorigenesis in this model...
2017: PloS One
https://www.readbyqxmd.com/read/28248728/clinicopathologic-features-of-the-non-cns-primary-ewing-sarcoma-family-of-tumors-in-the-head-and-neck-region
#9
Chang Gok Woo, Bora Lee, Joon Seon Song, Kyung-Ja Cho
Ewing sarcoma family of tumor (ESFT) is a group of malignant neoplasms that affect children and young adults. Primary ESFT does not commonly arise from the head and neck region. This study aimed to elucidate the clinicopathologic characteristics of ESFT of the head and neck region except for central nervous system primitive neuroectodermal tumors. Among the 207 cases of ESFT of the bone and soft tissue, diagnosed at Asan Medical Center during a 20-year period, 25 (12.1%) involved the head and neck region. Of those, 21 were available for histologic, immunohistochemical, and molecular studies...
February 28, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28217164/primary-intraspinal-extradural-primitive-neuroectodermal-tumor-a-rare-case
#10
Shrikant V Rege, Jitendra Tadghare, Harshad Patil, Sharadendu Narayan
Primitive neuroectodermal tumors (PNETs) are aggressive childhood malignancies and are difficult to treat. Primary intraspinal PNETs are rare. These patients have poor prognosis with short survival time even after surgery and chemoradiation. As there are no standard guidelines exist for the management of these tumors, a multidisciplinary approach has been employed with varying success. According to the review of literature, only few cases of primary intraspinal extradural PNETs have been reported. Herein, author has described a case of intraspinal, extradural PNET...
October 2016: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28210452/ewing-sarcoma-of-the-oral-cavity-a-review
#11
REVIEW
Maria Margaix-Muñoz, José Bagán, Rafael Poveda-Roda
OBJECTIVES: A review is made of the clinical, diagnostic, therapeutic and survival characteristics of Ewing sarcoma (ES) of the oral cavity. MATERIAL AND METHODS: A systematic literature search was carried out, with restrictions referred to time (1960-2014), language (English and Spanish) and type of study (case reports, letters, datasets, reviews). The following MeSH terms and boolean operators were used: Ewing AND Sarcoma AND [tongue, jaw, maxilla, cheek, condyle OR temporomandibular, floor AND mouth, gum OR gingiva, palate OR palatal, lip, uvula, head AND neck]...
February 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28191622/embryonal-tumor-with-multilayered-rosettes-in-a-3-year-old-girl-case-report
#12
Aparna Govindan, Muralikrishnan Vp, Jacob Paul Alapatt
Embryonal tumor with multilayered rosettes(ETMR) is a new entity in the group of Primitive Neuroectodermal tumors of the central nervous system. It combines histopathological features of neuroblastoma and ependymoblastoma. This tumor occurs mostly in the first five years of life. A hallmark cytogenetic abnormality at the chromosome locus 19q13.42 has been identified. The authors report a case of ETMR in the parietooccipital region in a 3 year old girl who presented with features of raised intracranial tension...
January 8, 2017: Turkish Neurosurgery
https://www.readbyqxmd.com/read/28180935/clinicopathological-features-and-microsurgical-outcomes-for-giant-pediatric-intracranial-tumor-in-60-consecutive-cases
#13
Ailing Guo, Vigneyshwar Suresh, Xianzhi Liu, Fuyou Guo
PURPOSE: Giant pediatric intracranial tumor (GPIT) remains to be a challenging disease with high morbidity and mortality. METHODS: The clinical data of 60 patients under 18 years of age operated on with GPIT (≥5 cm in diameter) were retrospectively analyzed. RESULTS: Gross total resection was achieved in 46 cases (77%) and subtotal resection was obtained in 14 cases (23%). Ninety percent (47/52) of the cases with obstructive hydrocephalus were resolved remarkably and only 10% (5/52) of the patients needed a ventriculoperitoneal shunt after tumor resection...
February 8, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28096196/craniospinal-irradiation-using-helical-tomotherapy-for-central-nervous-system-tumors
#14
Sanziana R I Schiopu, Gregor Habl, Matthias Häfner, Sonja Katayama, Klaus Herfarth, Juergen Debus, Florian Sterzing
The aim of this study was to describe early and late toxicity, survival and local control in 45 patients with primary brain tumors treated with helical tomotherapy craniospinal irradiation (HT-CSI). From 2006 to 2014, 45 patients with central nervous system malignancies were treated with HT-CSI. The most common tumors were medulloblastoma in 20 patients, ependymoma in 10 patients, intracranial germinoma (ICG) in 7 patients, and primitive neuroectodermal tumor in 4 patients. Hematological toxicity during treatment included leukopenia Grades 1-4 (6...
January 17, 2017: Journal of Radiation Research
https://www.readbyqxmd.com/read/28071641/epidemiological-data-and-case-load-spectrum-of-patients-presenting-to-bone-and-soft-tissue-disease-management-group-at-a-tertiary-cancer-center
#15
A Gulia, A Puri, S Chorge, P K Panda
INTRODUCTION AND BACKGROUND: This study was conducted to know the spectrum and number of bone and soft tissue (BST) tumors presenting to our institute. We needed to assess the gap between the number of patients seen and infrastructure available, and based on this information, help formulate guidelines for optimum utilization of resources and to provide best possible evidence-based cancer care. SETTINGS AND DESIGNS: This is a prospective observational study (epidemiological)...
April 2016: Indian Journal of Cancer
https://www.readbyqxmd.com/read/28060373/small-round-blue-cell-tumors-of-the-sinonasal-tract-a-differential-diagnosis-approach
#16
Lester Dr Thompson
One of the most challenging diagnostic categories within tumors of the sinonasal tract is the small round blue cell tumors. Biopsies are usually small and limited, resulting in considerable diagnostic difficulty for practicing surgical pathologists. These tumors share several overlapping histologic and immunophenotypic findings while also showing considerable variation within and between cases. Specific tumor site of origin, imaging findings, and clinical findings must be combined with the histology and pertinent ancillary studies if the correct diagnosis is to be reached...
January 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28039368/long-term-neurologic-health-and-psychosocial-function-of-adult-survivors-of-childhood-medulloblastoma-pnet-a-report-from-the-childhood-cancer-survivor-study
#17
Allison A King, Kristy Seidel, Chongzhi Di, Wendy M Leisenring, Stephanie Mabry Perkins, Kevin R Krull, Charles A Sklar, Daniel M Green, Gregory T Armstrong, Lonnie K Zeltzer, Elizabeth Wells, Marilyn Stovall, Nicole J Ullrich, Kevin C Oeffinger, Leslie L Robison, Roger J Packer
BACKGROUND: Medulloblastoma is the most common malignant childhood brain tumor, although long-term risks for chronic neurologic health and psychosocial functioning in aging adult survivors are incompletely characterized. METHODS: The Childhood Cancer Survivor Study (CCSS) includes 380 five-year survivors of medulloblastoma/primitive neuroectodermal tumor (PNET; median age at follow-up: 30 y, interquartile range 24-36) and sibling comparison (n = 4031). Cumulative incidence of neurologic health conditions was reported...
December 29, 2016: Neuro-oncology
https://www.readbyqxmd.com/read/28029420/early-dural-metastasis-from-a-case-of-glioblastoma-with-primitive-neuroectodermal-differentiation-a-case-report-and-literature-review
#18
Subhas K Konar, Shyamal C Bir, Tanmoy K Maiti, Devi Prasad Patra, Angela C DiPoto Brahmbhatt, Jamie A Jacobsohn, Anil Nanda
Glioblastoma with a primitive neuroectodermal (PNET) variant is a rare primary parenchymal tumor. Only a few cases of extraparenchymal metastasis are reported in world literature. Although the overall survival duration of glioblastoma multiforme (GBM) with primitive neuroectodermal tumor (PNET) variety may be prolonged in comparison to classical glioblastoma, the metastatic trend is completely different, and the prognosis is worse. We report an early dural metastasis of pure PNET component appearing in a case of primary glioblastoma with PNET variant...
January 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28017744/first-case-of-primary-sellar-suprasellar-intraventricular-ewing-sarcoma-case-report-and-review-of-the-literature
#19
Pier Paolo Mattogno, Davide Nasi, Corrado Iaccarino, Gabriele Oretti, Luisa Santoro, Antonio Romano
BACKGROUND: Intracranial Ewing sarcoma (ES) and peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare and poorly differentiated neoplasms. Immunohistochemical and cytogenetic findings support the possibility of a unique nosologic entity. Primary intracranial localization of this tumor is extremely rare; a few cases are reported in the literature, with only some confirmed by genetic studies. CASE DESCRIPTION: We report a 12-year-old patient with a sellar/suprasellar mass with intraventricular extension that in all its features mimicked a transinfundibular craniopharyngioma...
February 2017: World Neurosurgery
https://www.readbyqxmd.com/read/27984122/diagnostic-utility-of-cyclin-d1-in-the-diagnosis-of-small-round-blue-cell-tumors-in-children-and-adolescents
#20
Gaetano Magro, Lucia Salvatorelli, Rita Alaggio, Velia D'Agata, Ferdinando Nicoletti, Andrea Di Cataldo, Rosalba Parenti
Small round blue cell tumors (SRBCTs) of children and adolescents are often diagnostically challenging lesions. With the increasing diagnostic approach based on small biopsies, there is the need of specific immunomarkers that can help in the differential diagnosis among the different tumor histotypes to assure the patient a correct diagnosis for proper treatment. Based on our recent studies showing cyclin D1 overexpression in both Ewing's sarcoma/primitive peripheral neuroectodermal tumor (EWS/pPNET) and peripheral neuroblastic tumors (neuroblastoma and ganglioneuroblastoma), we immunohistochemically assessed cyclin D1 immunoreactivity in 128 cases of SRBCTs in children and adolescents in order to establish its potential utility in the differential diagnosis...
October 27, 2016: Human Pathology
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