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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/27923222/pediatric-primary-diffuse-leptomeningeal-primitive-neuroectodermal-tumor-a-case-report-and-literature-review
#1
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...
December 7, 2016: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27920700/primary-ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-stomach
#2
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
#3
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-pnet-arising-from-an-ovarian-mature-cystic-teratoma-in-a-12-year-old-female-a-case-report
#4
Ashley Jaramillo-Huff, Rania Bakkar, Jason Q McKee, Nancy Sokkary
BACKGROUND: Ovarian mature cystic teratomas (MCT) rarely transform to a primary primitive neuroectodermal tumor (PNET)(1). This case report offers evidence that MCTs may have undetected micro-focuses of malignant neural tumors. CASE: We describe the case of a 12-year-old female that presented with right-sided abdominal pain and distention. Intra-operative findings revealed a right ovarian MCT. However, pathology showed a 0.5 centimeter focus of malignant neural tumor within the 11 centimeter 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
#5
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
#6
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 tumour which was discovered by computed tomography. 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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/27781423/malignant-glioma-with-primitive-neuroectodermal-tumor-like-component-mg-pnet-novel-microarray-findings-in-a-pediatric-patient
#13
Jinglan Liu, Matthew P Keisling, Ayman Samkari, Gregory Halligan, Judy M Pascasio, Christos D Katsetos
Central nervous system (CNS) tumors exhibiting dual features of malignant glioma (MG) and primitive neuroectodermal tumor (PNET) are rare and diagnostically challenging. Previous studies have shown that MG-PNET carry MYCN or MYC gene amplifications within the PNET component concomitant with glioma-associated alterations, most commonly 10q loss, in both components [9]. Here we confirm and extend the profile of molecular genetic findings in a MG-PNET involving the left frontal lobe of a 12-year-old male. Histologically, the PNET-like component showed morphological features akin to anaplastic medulloblastoma highlighted by widespread immunoreactivity for βIII-tubulin (TUBB3) and nonphosphorylated neurofilament protein, and to a lesser degree, Neu-N, synaptophysin, and CD99, whereas the gliomatous component was demarcated by glial fibrillary acidic protein (GFAP) labeling...
October 26, 2016: Clinical Neuropathology
https://www.readbyqxmd.com/read/27778212/survival-after-chemotherapy-and-stem-cell-transplant-followed-by-delayed-craniospinal-irradiation-is-comparable-to-upfront-craniospinal-irradiation-in-pediatric-embryonal-brain-tumor-patients
#14
David R Raleigh, Bryan Tomlin, Benedict Del Buono, Erika Roddy, Katherine Sear, Lennox Byer, Erin Felton, Anu Banerjee, Joseph Torkildson, David Samuel, Biljana Horn, Steve E Braunstein, Daphne A Haas-Kogan, Sabine Mueller
Pediatric embryonal brain tumor patients treated with craniospinal irradiation (CSI) are at risk for adverse effects, with greater severity in younger patients. Here we compare outcomes of CSI vs. high-dose chemotherapy (HD), stem cell transplant (SCT) and delayed CSI in newly diagnosed patients. Two hundred one consecutive patients treated for medulloblastoma (72 %), supratentorial primitive neuroectodermal tumor (sPNET; 18 %) or pineoblastoma (10 %) at two institutions between 1988 and 2014 were retrospectively identified...
October 24, 2016: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/27776324/ewing-sarcoma-primitive-neuroectodermal-tumor-of-the-kidney-a-report-of-three-cases
#15
Maryam Abolhasani, Sareh Salarinejad, Mohammad Kazem Moslemi
INTRODUCTION: Ewing sarcoma/Primitive neuroectodermal tumor of the kidney (ES/PNET) is a member of Ewing's sarcoma family, occurring in young adults and has aggressive clinical behavior and poor prognosis. However, its discrimination from the renal cell carcinoma (RCC) is very difficult preoperatively. We present three cases of this rare disease that were managed in two academic centers. PRESENTATION OF CASES: Herein we report three cases of ES/PNET of the kidney, 2 young men complaining of right flank pain and gross hematuria and one young woman complaining of left subcostal pain...
October 18, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/27770281/long-term-safety-of-growth-hormone-replacement-therapy-after-childhood-medulloblastoma-and-pnet-it-is-time-to-set-aside-old-concerns
#16
Alice Indini, Elisabetta Schiavello, Veronica Biassoni, Luca Bergamaschi, Maria Chiara Magni, Nadia Puma, Stefano Chiaravalli, Federica Pallotti, Ettore Seregni, Barbara Diletto, Emilia Pecori, Lorenza Gandola, Geraldina Poggi, Maura Massimino
To assess the long-term safety of administering growth hormone (GH) in patients with GH deficiency due to treatment for childhood medulloblastoma and primitive neuroectodermal tumor (PNET). Data were retrospectively retrieved on children receiving GH supplementation, assessing their disease-free and overall survival outcomes and risk of secondary malignancies using Kaplan-Meier and Cox models. Overall 65 children were consecutively collected from May 1981 to April 2013. All patients had undergone craniospinal irradiation (total dose 18-39 Gy), and subsequently received GH for a median (interquartile range, IQR) of 81 (50...
October 21, 2016: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/27731566/askin-pnet-tumor-unmasked-by-trauma-in-a-young-male-patient
#17
Avas Chandra Ray, Subhra Aditya, Pulak Kumar Jana, Apratim Chatterjee, Anup Sarkar, Jay Mehta, Jotideb Mukhopadhyay
A young male labourer developed pain at the site of blunt trauma over back of chest followed by fever, cough with expectoration, breathlessness and hemorrhagic pleural effusion in the side of injury. What could have been passed as a sequel of trauma turned out to be the consequences of an underlying rare and aggressive malignant tumor of the chest wall known as Askin tumor or Primitive Neuroectodermal Tumor (PNET). CT thorax with guided FNAC, debulking operation, histopathological examination followed by immunohistochemistry of the tumor tissue led to the final diagnosis...
March 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27728624/case-of-mediastinal-ewing-s-sarcoma-primitive-neuroectodermal-tumor-presenting-as-pleural-effusion
#18
Snehal J, Suresh Sr
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27721314/gamma-knife-surgery-for-recurrent-or-residual-supratentorial-primitive-neuroectodermal-tumors
#19
Young-Bem Se, Dong Gyu Kim, Sung Kwon Kim, Jin Wook Kim, Yong Hwy Kim, Chul-Kee Park, Hyun-Tai Chung, Sun-Ha Paek
BACKGROUND: Supratentorial primitive neuroectodermal tumors (PNETs) are highly malignant and rare tumors of the central nervous system. OBJECTIVES: The aim of this study was to determine the role of Gamma Knife surgery (GKS) as a salvage treatment option for patients with recurrent or residual supratentorial PNETs. METHODS: Between 1998 and 2014, 11 patients with supratentorial PNETs were retrospectively analyzed. This series consisted of 7 male and 4 female patients...
2016: Stereotactic and Functional Neurosurgery
https://www.readbyqxmd.com/read/27703957/primary-renal-primitive-neuroectodermal-tumor-ewing-s-sarcoma-imaging-and-pathologic-findings-of-a-patient-with-a-nine-year-eight-month-disease-free-period-case-report-and-review-of-literature
#20
Ivan Zokalj, Jasminka Igrec, Antonio Plesnar
INTRODUCTION: Primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (EWS) belongs to a family of neoplasms that are presumed to originate from the neuroectodermal crest. PNET/EWSs are highly aggressive malignancies that usually present in the form of bone or soft tissue masses and usually affect adolescents and young adults. Primary PNET/EWS of the kidney is very rare. CASE PRESENTATION: We present the case of a 32-year-old female patient with primary renal PNET/EWS diagnosed nine years and eight months earlier...
July 2016: Nephro-urology Monthly
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