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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/28096196/craniospinal-irradiation-using-helical-tomotherapy-for-central-nervous-system-tumors
#1
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
#2
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
#3
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
#4
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
#5
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-literature
#6
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. Recent immunohistochemical and cytogenetic findings support the possibility of a unique nosological entity. Primary intracranial localization of this tumor is extremely rare: a small number of cases are reported in the literature, with only a part of them confirmed by genetic studies. CASE DESCRIPTION: We report the case of 12 year-old-patient affected by sellar-suprasellar mass with intraventricular extension that in all its features mimes a transinfundibular craniopharyngioma...
December 22, 2016: 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
#7
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
https://www.readbyqxmd.com/read/27974690/benefit-of-sunitinib-in-the-treatment-of-pulmonary-primitive-neuroectodermal-tumors-a-case-report-and-literature-review
#8
Chunhui Zhang, Jingchun Zhang, Guangyu Wang, Jiajia Xu, Yanlin Li, Qing Guo, Tongsen Zheng, Yanqiao Zhang
Primitive neuroectodermal tumor (PNET) is a highly aggressive small round celltumor but is extremely rare in the lung. Next-generation sequencing (NGS) has led to breakthroughs for genetic analyses and personalizedmedicine approaches for cancer treatment.We report the case of a 30-year-old woman with an advanced pulmonary PNET treated with multiple chemotherapeutic regimens, and achieved a partial response (PR) as a best response. However, there was a disease progression after these treatment regimens.The NGS revealed the presence of a copy number loss (CNL) of Von Hippel-Lindau (VHL), CDKN2A/B and TP53 genes...
December 10, 2016: Oncotarget
https://www.readbyqxmd.com/read/27923222/pediatric-primary-diffuse-leptomeningeal-primitive-neuroectodermal-tumor-a-case-report-and-literature-review
#9
Jesna Mathew Sublett, Caitlin Davenport, Howard Eisenbrock, Shamsher Dalal, Syed A Jaffar Kazmi, Amir Kershenovich
BACKGROUND: Primary diffuse leptomeningeal primitive neuroectodermal tumor (PDL PNET) is extremely rare, with only 19 cases reported in the literature to date. We present a case of a child with rapidly progressive PDL PNET and a literature review. A 10-year-old boy presented with mood lability, hallucinations, generalized pain, enuresis, and headaches. Initial investigation failed to produce a diagnosis. The symptoms progressed to seizure, back pain, and papilledema. Imaging showed acute hydrocephalus and mild diffuse leptomeningeal enhancement without an identifiable primary lesion...
7, 2016: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27920700/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-stomach
#10
Safi Khuri, Hayim Gilshtein, Sa'd Sayidaa, Bishara Bishara, Yoram Kluger
Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET...
September 2016: Case Reports in Oncology
https://www.readbyqxmd.com/read/27920284/a-giant-dumbbell-shaped-primitive-neuroectodermal-tumor-in-the-brain
#11
Zhi Gang Lan, Mao Jun Chen, Jin Li, Yanhui Liu
No abstract text is available yet for this article.
December 6, 2016: Neurology
https://www.readbyqxmd.com/read/27919713/primary-primitive-neuroectodermal-tumor-arising-from-an-ovarian-mature-cystic-teratoma-in-a-12-year-old-girl-a-case-report
#12
Ashley Jaramillo-Huff, Rania Bakkar, Jason Q McKee, Nancy Sokkary
BACKGROUND: Ovarian mature cystic teratomas (MCTs) rarely transform to primary primitive neuroectodermal tumors. This case report offers evidence that MCTs might have undetected microfoci of malignant neural tumors. CASE: We describe the case of a 12-year-old girl who presented with right-sided abdominal pain and distention. Intraoperative findings revealed a right ovarian MCT. However, pathology showed a 0.5-cm focus of malignant neural tumor within the 11-cm MCT...
December 2, 2016: Journal of Pediatric and Adolescent Gynecology
https://www.readbyqxmd.com/read/27916050/-the-clinical-and-pathological-features-of-85-cases-with-positive-cerebrospinal-fluid-cytology-by-thin-layer-centrifugal-cytological-test
#13
W Gao, Z Li, L M Wang, F Lian, C C Liu, D H Lu, Y S Piao
Objective: To evaluate cytological test of cerebrospinal fluid in the diagnosis of meningeal dissemination of tumor cells. Methods: The clinical and imaging features of 85 cases with tumor cells diagnosed by thin-layer centrifugal cytological test of cerebrospinal fluid were retrospectively reviewed. The characteristics of cellular morphology and immunocytochemical staining were analyzed. Results: The main presentations of all the patinets was meningeal irritation and neurological dysfunction. The features of the brain MRI were meningeal thicking and enhancement, intracranial abnormal signals and intracranial space occupying lesion in part of the patients...
December 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27905830/isolated-cardiac-peripheral-primitive-neuroectodermal-tumor-a-case-report
#14
Chengming Fan, Demiao Kong, Changming Tan, Jinfu Yang
BACKGROUND: Peripheral primitive neuroectodermal tumor isolated in the heart, presenting as a primary cardiac tumor is considered as extremely rare. METHODS: We present a 53-year-old Chinese female with a cardiac tumor which was discovered by CT. RESULTS: A hypo-intense tumorous mass was shown extending from the left ventricle by Cardiac CT, and fused FDG positron emission tomography demonstrated no other abnormal FDG active lesions in the body...
December 1, 2016: Cancer Biology & Therapy
https://www.readbyqxmd.com/read/27878756/imaging-findings-of-adrenal-primitive-neuroectodermal-tumors-a-series-of-seven-cases
#15
Y Zhang, P Cai, M Chen, X Yi, L Li, D Xiao, W Liu, W Li, Y Li
OBJECTIVE: To explore the imaging features of adrenal primitive neuroectodermal tumors (PNETs). MATERIALS AND METHODS: This retrospective study included seven patients with surgically and pathologically confirmed adrenal PNETs. Among them, six underwent computed tomography (CT) scans, and one underwent magnetic resonance imaging. The imaging findings, including size, shape, margin, hemorrhage, calcification, cystic degeneration, regional lymph nodes involvement, tumor thrombus formation and enhancement pattern, were retrospectively analyzed...
November 23, 2016: Clinical & Translational Oncology
https://www.readbyqxmd.com/read/27822490/primitive-neuroectodermal-tumor-of-the-uterus
#16
C R Elizalde, A Yagüe, J Fernandez, P Dieste, M J Puente, J Hernandez
•PNET of the uterus is rare and requires early diagnoses and treatment.•Molecular analysis is important to distinguish it from other tumors.•Different combinations of adjuvant chemotherapy have been report.
November 2016: Gynecologic Oncology Reports
https://www.readbyqxmd.com/read/27818821/disseminated-cerebrospinal-embryonal-tumor-in-the-adult
#17
Alessandro Caporlingua, Daniele Armocida, Federico Caporlingua, Gennaro Lapadula, Grazia Maria Elefante, Manila Antonelli, Maurizio Salvati
Introduction. According to the 2016 World Health Organization classification of Tumors of the Central Nervous System, the term Primitive Neuroectodermal Tumor has been replaced by the term Embryonal Tumor (ET). We present a case of disseminated cerebrospinal ET presenting in an adult patient. Illustrative Case. A 49-year-old male presenting with low back pain, dysuria, and hypoesthesia of the lower extremities referred to our emergency department. Brain and whole spine contrast-enhanced MRI documented a diffusively disseminated heterogeneous neoplasm with intradural extra- and intramedullary involvement of the cervicothoracic tract and cauda equina...
2016: Case Reports in Pathology
https://www.readbyqxmd.com/read/27804208/birth-weight-and-subsequent-risk-of-childhood-primary-brain-tumors-an-updated-meta-analysis
#18
Anne Dahlhaus, Peggy Prengel, Logan Spector, Dawid Pieper
BACKGROUND: Primary brain tumors are common in childhood, but the etiology is largely unclear. As studies on birth weight as a risk factor for the occurrence of histologically specified tumors have been inconclusive, we decided to update a 2008 meta-analysis on the subject. METHODS: A search strategy was performed in Medline and EMBASE for the period 2007-2016. We included six new studies and performed further subgroup analyses for medulloblastoma and primitive neuroectodermal tumors (PNETs)...
November 2, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27784193/successful-management-of-a-patient-with-malignant-thyroid-teratoma
#19
Guilherme Rabinowits, Justine Barletta, Lynette M Sholl, Encarnacion Reche, Jochen Lorch, Laura Goguen
BACKGROUND: Malignant thyroid teratomas are rare tumors with a poor prognosis. Little is known about their pathogenesis or treatment. Here, the case is reported of an adult woman with an aggressive thyroid teratoma with primitive neuroectodermal tumor (PNET) malignant transformation, successfully managed with neoadjuvant chemotherapy and surgery. PATIENT FINDINGS: Sequencing of paired tumor and normal tissues revealed a DICER1 c.5438A>G (p.E1813G) somatic mutation in 56% of sequencing reads consistent with a driver event...
November 28, 2016: Thyroid: Official Journal of the American Thyroid Association
https://www.readbyqxmd.com/read/27783941/mek-inhibitors-reverse-growth-of-embryonal-brain-tumors-derived-from-oligoneural-precursor-cells
#20
Katarzyna Modzelewska, Elena F Boer, Timothy L Mosbruger, Daniel Picard, Daniela Anderson, Rodney R Miles, Mitchell Kroll, William Oslund, Theodore J Pysher, Joshua D Schiffman, Randy Jensen, Cicely A Jette, Annie Huang, Rodney A Stewart
Malignant brain tumors are the leading cause of cancer-related deaths in children. Primitive neuroectodermal tumors of the CNS (CNS-PNETs) are particularly aggressive embryonal tumors of unknown cellular origin. Recent genomic studies have classified CNS-PNETs into molecularly distinct subgroups that promise to improve diagnosis and treatment; however, the lack of cell- or animal-based models for these subgroups prevents testing of rationally designed therapies. Here, we show that a subset of CNS-PNETs co-express oligoneural precursor cell (OPC) markers OLIG2 and SOX10 with coincident activation of the RAS/MAPK (mitogen-activated protein kinase) pathway...
October 25, 2016: Cell Reports
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