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primitive neuroectodermal tumor

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https://www.readbyqxmd.com/read/28515922/primary-primitive-neuroectodermal-tumor-of-the-cervix-a-report-of-two-cases-and-review-of-the-literature
#1
Xiaofeng Wang, Yan Gao, Yanning Xu, Yixin Liu, Pengpeng Qu
Peripheral primitive neuroectodermal tumor (PNET) of the cervix uteri is extremely rare. Two cases of PNET of the cervix are presented herein. Two female patients, aged 48 and 43 years, presented with irregular uterine bleeding over the course of 1 year, and increased urinary frequency for 1 month, respectively. On gynecological examination, a mass in the cervix was palpated and a biopsy performed. The findings of the initial biopsy revealed small-cell carcinoma in both patients. Following neoadjuvant chemotherapy and radiotherapy, radical hysterectomy was performed in both patients...
May 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28510278/tfg-met-fusion-in-an-infantile-spindle-cell-sarcoma-with-neural-features
#2
Uta Flucke, Max M van Noesel, Marc Wijnen, Lei Zhang, Chun-Liang Chen, Yun-Shao Sung, Cristina R Antonescu
An increasing number of congenital and infantile sarcomas displaying a primitive, monomorphic spindle cell phenotype have been characterized to harbor recurrent gene fusions, including infantile fibrosarcoma and congenital spindle cell rhabdomyosarcoma. Here we report an unusual spindle cell sarcoma presenting as a large and infiltrative pelvic soft tissue mass in a 4-month-old girl, which revealed a novel TFG-MET gene fusion by whole transcriptome RNA sequencing. The tumor resembled the morphology of an infantile fibrosarcoma with both fascicular and patternless growth, however, it expressed strong S100 protein immunoreactivity, while lacking SOX10 staining and retaining H3K27me3 expression...
May 16, 2017: Genes, Chromosomes & Cancer
https://www.readbyqxmd.com/read/28509141/primary-ewing-sarcoma-of-the-kidney-a-case-report-and-treatment-review
#3
Muhammad Sadiq, Iftikhar Ahmad, Jamila Shuja, Khushnaseeb Ahmad
Ewing sarcomas/primitive neuroectodermal tumors (ES/PNET) of the kidney are rarely found high-grade malignant tumors, offering poor prognosis. Although established treatment guidelines for ES of kidney are scarce, a multi-modality treatment approached is typically implemented. Herein, we report a 14-year-old female patient with ES of right kidney. Post-nephrectomy disease recurrence was treated with chemotherapy (i.e., vincristine, doxorubicin and cyclophosphamide); marked reduction in tumor size (i.e., from 18...
April 21, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508928/embryonal-tumors-in-canadian-children-less-than-36-months-of-age-results-from-the-canadian-pediatric-brain-tumor-consortium-cpbtc
#4
E Story, D L Johnston, U Bartels, A S Carret, B Crooks, D D Eisenstat, C Fryer, L Lafay-Cousin, V Larouche, B Wilson, S Zelcer, M Silva, J Brossard, E Bouffet, D L Keene
Embryonal tumors are a heterogeneous group of central nervous system (CNS) tumors whose subgroups have varying incidence and outcome. Despite these differences, they are often grouped as a single entity for study purposes. To date, there are no Canadian multi-institutional studies examining the incidence and outcome of all embryonal subtypes. The current study is an observational study reviewing embryonal tumors in all patients less than 36 months of age diagnosed with a CNS tumor in Canada from 1990 to 2005...
May 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28472972/primary-ewing-s-sarcoma-primitive-neuroectodermal-tumor-of-the-ileum-case-report-of-a-16-year-old-chinese-female-and-literature-review
#5
Teng Li, Fang Zhang, Yarui Cao, Shoubin Ning, Yongmin Bi, Weicheng Xue, Li Ren
BACKGROUND: Ewing's sarcoma (ES) and primitive neuroectodermal tumors (PNET) are closely related tumors. Although soft tissue ES/PNET are common in clinical practice, they are rare in the small intestine. Because of the absence of characteristic clinical symptoms, they are easily misdiagnosed as other benign or malignant diseases. CASE PRESENTATION: Here, we present the case of a 16-year-old female who complained of anemia and interval hematochezia. Her serum test results showed only a slight elevation of CA-125 and a low level of hemoglobin...
May 4, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28469346/primitive-neuroectodermal-tumor-of-lung-in-adult-with-hemorrhagic-brain-metastasis-an-extremely-rare-case-scenario
#6
Abhishek Purkayastha, Neelam Sharma, Amul Kapur, Kavita Sahai
Primitive neuroectodermal tumors (PNETs) are highly malignant neoplasms of embryonal origin manifesting in children and adolescents, rarely seen in adults. Carcinoma lung with hemorrhagic metastasis to the brain is very common, but primary lung PNET with hemorrhagic brain metastasis is extremely uncommon. We hereby report a 29-year-old female diagnosed as PNET lung was treated with vincristine, adriamycin, and cyclophosphamide alternating with ifosfamide plus etoposide followed by radiotherapy (RT). After 9 months, she developed hemorrhagic brain metastasis from PNET lung confirmed from tissue immunohistology postcraniotomy...
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28469330/profile-of-primary-pediatric-brain-and-spinal-cord-tumors-from-north-india
#7
Nadia Shirazi, Meenu Gupta, Nowneet Kumar Bhat, Braham Prakash Kalra, Ranjit Kumar, Manju Saini
BACKGROUND AND OBJECTIVE: The study was carried out to find the profile of pediatric brain and spinal cord tumors during 2006-2015 in a tertiary referral center of North India. MATERIALS AND METHODS: It was a retrospective medical record-based observational study. All children <18 years of age with confirmed histopathological diagnosis of cancer were included in the study. RESULTS: Central nervous system (CNS) tumors constituted 5.6% of all pediatric solid malignancies in our hospital...
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28468165/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...
May 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28462848/outcome-of-resectable-pediatric-ewing-sarcoma-of-the-ribs
#9
Gehad Ahmed, Manal Zamzam, Mohamed S Zaghloul, Ahmed Kamel, Ranin Soliman, Iman Zaky, Asmaa Salama, Nehal Kamal, Maged ElShafiey
PURPOSE: Was to evaluate the outcome of multimodality treatment in resectable primary Ewing sarcoma/primitive neuroectodermal tumor ES/PNET of the ribs and role of thoracoscopy in facilitating resection of these tumors. PATIENTS AND METHODS: This was a retrospective study including 22 patients with primary ES/PNET of the ribs surgically treated at Children's Cancer Hospital Egypt (CCHE) between January 2008 until the end of December 2014. RESULTS: Median age was 8...
April 24, 2017: Journal of the Egyptian National Cancer Institute
https://www.readbyqxmd.com/read/28459463/mycn-induces-neuroblastoma-in-primary-neural-crest-cells
#10
R R Olsen, J H Otero, J García-López, K Wallace, D Finkelstein, J E Rehg, Z Yin, Y-D Wang, K W Freeman
Neuroblastoma (NBL) is an embryonal cancer of the sympathetic nervous system (SNS), which causes 15% of pediatric cancer deaths. High-risk NBL is characterized by N-Myc amplification and segmental chromosomal gains and losses. Owing to limited disease models, the etiology of NBL is largely unknown, including both the cell of origin and the majority of oncogenic drivers. We have established a novel system for studying NBL based on the transformation of neural crest cells (NCCs), the progenitor cells of the SNS, isolated from mouse embryonic day 9...
May 1, 2017: Oncogene
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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