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Primitive Neuroectodermal Tumors of the Central Nervous System

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Genomic Molecular Classification of CNS Malignancies.

Marissa Spino, Matija Snuderl,

Molecular studies have identified distinct genomic drivers providing insights in biology of brain tumors. Advances in genetic and epigenetic analysis, as well as development of mutation-specific antibodies enable more accurate classification of histologically indistinguishable tumors. Compared with histopathologic grading, molecular biomarkers are also superior in predicting natural behavior of tumors ... Read more >>

Adv Anat Pathol (Advances in anatomic pathology)
[2020, 27(1):44-50]

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Management of Ewing Sarcoma Family of Tumors: A Short Description of a Rare Primitive Uterine pPNET and Literature Review.

Salvatore Pisconti, Giuseppina Della Vittoria Scarpati, Carlo Buonerba, Simona Messinese, Roberta Carella, Massimiliano Di Marzo, Giuseppe Di Lorenzo, Grazia Lazzari, Francesco Perri, Raffaele Solla,

Purpose:To describe the outcome of a patient with a rare primitive uterine pPNET and to perform a review of the available data in literature, leading the clinicians to better face this rare disease. Methods:We have rescued data regarding the multidisciplinary treatment of pPNET from the PUBMED database, highlighting also issues ... Read more >>

Onco Targets Ther (OncoTargets and therapy)
[2020, 13:1179-1184]

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Salvage therapy for progressive, treatment-refractory or recurrent pediatric medulloblastoma: a systematic review protocol.

Ashley A Adile, Michelle M Kameda-Smith, David Bakhshinyan, Laura Banfield, Sabra K Salim, Forough Farrokhyar, Adam J Fleming,

BACKGROUND:Central nervous system tumors remain the leading cause of cancer-related mortality amongst children with solid tumors, with medulloblastoma (MB) representing the most common pediatric brain malignancy. Despite best current therapies, patients with recurrent MB experience have an alarmingly high mortality rate and often have limited therapeutic options beyond inadequate chemotherapy ... Read more >>

Syst Rev (Systematic reviews)
[2020, 9(1):47]

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Population-Based Analysis of Demographic and Socioeconomic Disparities in Pediatric CNS Cancer Survival in the United States.

Robert Fineberg, Shadi Zahedi, Megan Eguchi, Muriel Hart, Myles Cockburn, Adam L Green,

Previous studies have demonstrated effects of racial and socioeconomic factors on survival of adults with cancer. While less studied in the pediatric population, data exist demonstrating disparities of care and survival in pediatric oncology patients based on socioeconomic and racial/ethnic factors. Brain cancers recently overtook leukemia as the number one ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):4588]

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Therapeutic strategies for uncommon testis cancer histologies: teratoma with malignant transformation and malignant testicular sex cord stromal tumors.

Mounsif Azizi, Ahmet M Aydin, Salim K Cheriyan, Charles C Peyton, Matthew Montanarella, Scott M Gilbert, Wade J Sexton,

Testicular cancer is the most common solid malignancy in male adolescents and young adults, with germ cell derived seminomas and non-seminomas being by far the most common histologies. Teratoma with somatic-type malignancy is a rare chemo-resistant phenotype of testis cancer associated with poor prognosis in patients with advanced stage disease. ... Read more >>

Transl Androl Urol (Translational Andrology and Urology)
[2020, 9(Suppl 1):S91-S103]

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A Practical Approach to the Differential Diagnosis of Intracranial Tumors: Gross, Histology, and Immunoprofile-based Algorithm.

George S Stoyanov, Lilyana Petkova, Deyan L Dzhenkov,

Intracranial tumors are a diverse group of conditions, both benign and malignant, primary and metastatic and always require detailed medical information, radiological reports and deep knowledge of the histological hallmarks and immunohistochemical profile of different types of tumors and tumor-like processes. Despite it clinically often being possible to differentiate between ... Read more >>

Cureus (Cureus)
[2019, 11(12):e6384]

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ATRT of lateral ventricle in a child: A Rare Tumor at a Very Rare Location.

Fayçal Lakhdar, Mohammed Benzagmout, Yasser Arkha, Khalid Chakour, Mohammed El Faiz Chaoui,

Atypical teratoid/rhabdoid tumors (AT/RTs) of infancy are highly malignant central nervous system neoplasms that are most commonly seen during the first 2 years of life with limited therapeutic options. To date, only two cases have been described in the lateral ventricle. A 4-year-old boy presented with a 4-month history of ... Read more >>

Asian J Neurosurg (Asian journal of neurosurgery)
[2020, 15(1):225-229]

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Circulating leptin levels are associated with adiposity in survivors of childhood brain tumors.

E Danielle Sims, William J Jennings, Brianna Empringham, Adam Fleming, Carol Portwine, Donna L Johnston, Shayna M Zelcer, Shahrad Rod Rassekh, Sarah Burrow, Lehana Thabane, M Constantine Samaan,

Survivors of Childhood Brain Tumors (SCBT) are at a higher risk of developing cardiovascular disease and type 2 diabetes compared to the general population. Adiposity is an important risk factor for the development of these outcomes, and identifying biomarkers of adiposity may help the stratification of survivors based on their cardiovascular ... Read more >>

Sci Rep (Scientific reports)
[2020, 10(1):4711]

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Mortality patterns in long-term survivors of childhood or adolescent central nervous system tumour in Sweden.

Wuqing Huang, Jan Sundquist, Kristina Sundquist, Jianguang Ji,

PURPOSE:A growing number of young patients with central nervous system (CNS) tumour survived for more than five years. However, these long-term survivors might be at risk of multiple late effects thus leading to a higher risk of late mortality. We aimed to explore the risk of late mortality and the ... Read more >>

J. Neurooncol. (Journal of neuro-oncology)
[2019, 145(3):541-549]

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Peripheral primitive neuroectodermal tumors: A retrospective analysis of 89 cases and literature review.

Liming Gao, Yingying Zhu, Xiaohua Shi, Zhiqiang Gao, Xingming Chen,

Peripheral primitive neuroectodermal tumors (pPNETs) are rare, small cell carcinomas with a poor prognosis. The aim of the present study was to describe therapeutic approaches, outcomes and probable prognostic factors. The clinical features, treatments, and outcomes of 89 consecutive patients with pPNET treated at the Peking Union Medical College Hospital ... Read more >>

Oncol Lett (Oncology letters)
[2019, 18(6):6885-6890]

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Medulloblastoma in a 6 Year Old Mixed Breed Dog: Surgical Debulking and Chemotherapy.

Rachel Lampe, Miranda D Vieson, Devon Hague, Dana Connell, Kari Foss, Kim A Selting,

A medulloblastoma was surgically debulked from a 6 year old American Staffordshire Terrier, who then received a modified lomustine (CCNU), vincristine, procarbazine, and prednisolone (LOPP) protocol. The dog improved significantly and continued to do well until deterioration and euthanasia 5 months following surgery. This is the first known published case ... Read more >>

Front Vet Sci (Frontiers in veterinary science)
[2019, 6:401]

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Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature.

Qiong-Qian Xu, Wen-Wen Xing, Gang Chen, Yi-Wu Dang, Yi-Ge Luo, Peng Chen, Song-Wu Liang, Jia-Bo Chen,

BACKGROUND:Primitive neuroectodermal tumors are rare, highly malignant small round cell tumors belonging to the Ewing sarcoma family. The purpose of this article is to present clinical manifestation, histology, treatment, and prognosis of two primitive neuroectodermal tumors (PNETs) in extremely rare anatomic locations, the abdominal wall and vulva. CASE SUMMARY:Case 1 ... Read more >>

World J Clin Cases (World journal of clinical cases)
[2019, 7(21):3671-3682]

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Embryonal tumor with multilayered rosettes, C19MC-altered (ETMR): a newly defined pediatric brain tumor.

Yu-Chun Pei, Guo-Hao Huang, Xiao-Hong Yao, Xiu-Wu Bian, Fei Li, Yan Xiang, Lin Yang, Sheng-Qing Lv, Jun Liu,

Embryonal tumor with multilayered rosettes (ETMR), C19MC-altered, is a newly defined and rare pediatric malignant tumor of the central nervous system (CNS) in the 2016 WHO Classification of Tumors of the Central Nervous System. Here we present two cases of ETMR with amplification of the C19MC locus at chromosome 19q13.42. ... Read more >>

Int J Clin Exp Pathol (International journal of clinical and experimental pathology)
[2019, 12(8):3156-3163]

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Phase II study of temozolomide and topotecan (TOTEM) in children with relapsed or refractory extracranial and central nervous system tumors including medulloblastoma with post hoc Bayesian analysis: A European ITCC study.

Gwénaël Le Teuff, Alicia Castaneda-Heredia, Christelle Dufour, Timothy Jaspan, Raphael Calmon, Annick Devos, Kieran McHugh, Pierre Leblond, Didier Frappaz, Isabelle Aerts, Christian M Zwaan, Stéphane Ducassou, Pascal Chastagner, Arnauld Verschuur, Nadège Corradini, Michela Casanova, Hervé Rubie, Riccardo Riccardi, Marie-Cecile Le Deley, Gilles Vassal, Birgit Geoerger, ,

AIM:To assess objective response after two cycles of temozolomide and topotecan (TOTEM) in children with refractory or relapsed miscellaneous extracranial solid and central nervous system (CNS) tumors, including medulloblastoma and primitive neuroectodermal tumors (PNET). PROCEDURE:Multicenter, nonrandomized, phase 2 basket trial including children with solid tumors, completed by a one-stage design ... Read more >>

Pediatr Blood Cancer (Pediatric blood & cancer)
[2020, 67(1):e28032]

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Morphological and immunophenotypic characterization of perivascular interstitial cells in human glioma: Telocytes, pericytes, and mixed immunophenotypes.

Lubov Mitrofanova, Anton Hazratov, Boris Galkovsky, Andrey Gorshkov, Danila Bobkov, Dmitry Gulyaev, Evgeny Shlyakhto,

Telocytes (Tcs) and pericytes (Pcs) are two types of perivascular interstitial cell known to be widespread in various organs and tissues, including the brain. We postulated that Tcs and Pcs may be involved in glioblastoma (GBM) neovascularization. Objective:Morphological study of Tc and Pc roles in GBM. Materials and Methods:Samples from ... Read more >>

Oncotarget (Oncotarget)
[2020, 11(4):322-346]

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ZBTB16: A new biomarker for primitive neuroectodermal tumor element / Ewing sarcoma.

Guang-Qian Xiao, Andy E Sherrod, Kyle M Hurth,

Primitive neuroectodermal tumor (PNET) traditionally encompasses two different classes of tumors with similar morphology - PNET of the peripheral nervous system (pPNET) and PNET of the central nervous system (cPNET). The latter also includes germ cell tumor-derived PNET (gPNET). There are currently no specific markers for gPNET. This study seeks ... Read more >>

Pathol. Res. Pract. (Pathology, research and practice)
[2019, 215(10):152536]

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Infratentorial Glioblastoma Metastasis to Bone.

Jocelyn A Ricard, Samuel W Cramer, River Charles, Carolina Gil Tommee, An Le, W Robert Bell, Clark C Chen, Margaret E Flanagan,

BACKGROUND:Glioblastoma multiforme (GBM) is a rapid-growing central nervous system neoplasm. We report a case of GBM with extensive intramedullary lumbar drop metastasis and highly unusual osseous spine metastasis from a primary infratentorial GBM occurring 10 years after the initial diagnosis, which to our knowledge has not been described previously. CASE ... Read more >>

World Neurosurg (World neurosurgery)
[2019, 131:90-94]

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Primitive neuroectodermal tumor of the esophagus with metastasis in the pineal gland.

Leonardo Blas Jhon, Paloma Sánchez-Fayos, Maria Jesus Martín Relloso, Daniel Calero Barón, Juan Carlos Porres Cubero,

Primitive neuroectodermal tumors (PNET) are very rare tumors that belong to a family of malignant neoplasms of tiny round cells which are derived from the neural crest. This report discusses a rare case of an adult woman with esophageal PNET, confirmed by immunohistochemistry, that presented with metastasis to the pineal ... Read more >>

Endosc Int Open (Endoscopy international open)
[2019, 7(9):E1163-E1165]

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Embryonal Rhabdomyosarcoma - A Mimicker of Squamosal Otitis Media.

Ajay Bhandarkar, Architha Menon, Ranjini Kudva, Kailesh Pujary,

Introduction:Rhabdomyosarcoma is the most frequently occurring intrusive soft tissue sarcoma in the pediatric age group. Orbit is the most common location for a pediatric rhabdomyosarcoma, but it can occur in the oral cavity, pharynx, face and neck in the descending order of incidence. Rhabdomyosarcoma in the ear is extremely rare. ... Read more >>

Iran J Otorhinolaryngol (Iranian journal of otorhinolaryngology)
[2020, 32(108):57-61]

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WHO grade has no prognostic value in the pediatric high-grade glioma included in the HERBY trial.

Pascale Varlet, Gwénaël Le Teuff, Marie-Cécile Le Deley, Felice Giangaspero, Christine Haberler, Thomas S Jacques, Dominique Figarella-Branger, Torsten Pietsch, Felipe Andreiuolo, Christophe Deroulers, Tim Jaspan, Chris Jones, Jacques Grill,

BACKGROUND:The World Health Organization (WHO) adult glioma grading system is questionable in pediatric high-grade gliomas (pHGGs), which are biologically distinct from adult HGGs. We took advantage of the neuropathological review data obtained during one of the largest prospective randomized pHGG trials, namely HERBY (NCT01390948), to address this issue in children ... Read more >>

Neuro-oncology (Neuro-oncology)
[2020, 22(1):116-127]

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Worldwide Trends in Survival From Common Childhood Brain Tumors: A Systematic Review.

Fabio Girardi, Claudia Allemani, Michel P Coleman,

PURPOSE:The histology of brain tumors determines treatment and predicts outcome. Population-based survival reflects the effectiveness of a health care system in managing cancer. No systematic review of worldwide variation and time trends in survival from brain tumors in children is currently available. PATIENTS AND METHODS:We considered longitudinal, observational studies comprising ... Read more >>

J Glob Oncol (Journal of global oncology)
[2019, 5:1-25]

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Do Glioblastomas with Syndromic Association Have Better Prognosis? A Case of Supratentorial Glioblastoma with Embryonal Tumor Differentiation in a Child with Multiple Enchondromatosis.

Siddharth Vankipuram, Sushant Sahoo, Shalini Bhalla, Chittij Srivastava,

Glioblastoma (GBM) is an aggressive cancerous neoplasm of the brain that has numerous morphological subtypes. Primitive neuroectodermal differentiation (hereafter, referred to as embryonal tumor [ET] differentiation) in GBM is one of them and is known to occur in adults. Their presentation in pediatric population is rare and can be a ... Read more >>

J Pediatr Neurosci (Journal of pediatric neurosciences)
[2019, 14(4):228-231]

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Anaplastic pleomorphic xanthoastrocytoma associated with an H3G34 mutation: a case report with review of literature.

Shoh Sasaki, Ran Tomomasa, Sumihito Nobusawa, Junko Hirato, Tomoko Uchiyama, Eishu Boku, Toshiteru Miyasaka, Takanori Hirose, Chiho Ohbayashi,

Here, we report a rare case of anaplastic pleomorphic xanthoastrocytoma (PXA) associated with an H3G34 mutation. A 12-year-old male presented with loss of appetite, vomiting, headache, and a generalized seizure, and CT revealed a 9.0 cm left frontal lobe mass with some septal walls and a localized high-density area suggestive of ... Read more >>

Brain Tumor Pathol (Brain tumor pathology)
[2019, 36(4):169-173]

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Evaluating Surgical Resection Extent and Adjuvant Therapy in the Management of Gliosarcoma.

Michael C Jin, Elisa K Liu, Siyu Shi, Iris C Gibbs, Reena Thomas, Lawrence Recht, Scott G Soltys, Erqi L Pollom, Steven D Chang, Melanie Hayden Gephart, Seema Nagpal, Gordon Li,

Introduction: Gliosarcomas are clinically aggressive tumors, histologically distinct from glioblastoma. Data regarding the impact of extent of resection and post-operative adjuvant therapy on gliosarcoma outcomes are limited. Methods: Patients with histologically confirmed gliosarcoma diagnosed between 1999 and 2019 were identified. Clinical, molecular, and radiographic data were assembled based on historical ... Read more >>

Front Oncol (Frontiers in Oncology)
[2020, 10:337]

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Prenatal Presentation of Medulloepithelioma: Case and Literature Review.

Nidhi Arora, Chanchal Ahmad, Arpit Gupta, Nitin Ghonge, Anita Kaul,

Congenital brain tumors (CBTs) are extremely rare and account for only 0.5%-1.9% of all pediatric brain tumors. Medulloepithelioma is one of the rare tumors with an incidence of about 1% among all CBTs with a very dismal prognosis and typically diagnosed at the median age of 24 months. The objective ... Read more >>

Cureus (Cureus)
[2019, 11(6):e5018]

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