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https://www.readbyqxmd.com/read/28074148/apraxia-of-speech-and-cerebellar-mutism-syndrome-a-case-report
#1
E De Witte, I Wilssens, D De Surgeloose, G Dua, M Moens, J Verhoeven, M Manto, P Mariën
BACKGROUND: Cerebellar mutism syndrome (CMS) or posterior fossa syndrome (PFS) consists of a constellation of neuropsychiatric, neuropsychological and neurogenic speech and language deficits. It is most commonly observed in children after posterior fossa tumor surgery. The most prominent feature of CMS is mutism, which generally starts after a few days after the operation, has a limited duration and is typically followed by motor speech deficits. However, the core speech disorder subserving CMS is still unclear...
2017: Cerebellum & Ataxias
https://www.readbyqxmd.com/read/28013334/cerebellopontine-angle-medulloblastoma-with-extensive-nodularity-in-a-child-case-report-and-review-of-the-literature
#2
Raywat Noiphithak, Vich Yindeedej, Chatchai Thamwongskul
BACKGROUND: Cerebellar medulloblastomas (MBs) are one of the most common posterior fossa tumors in children but rarely occur in the cerebellopontine angle (CPA). Only 39 cases of CPA MBs were reported in the literature, and most of them were classic and desmoplastic MBs. CASE PRESENTATION: A 22-month-old girl presented with progressive cerebellar ataxia. Magnetic resonance imaging showed a large tumor in the right CPA and obstructive hydrocephalus. Surgical resection was performed and achieved total tumor removal...
December 24, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28000417/a-phase-ii-feasibility-study-of-oral-etoposide-given-concurrently-with-radiotherapy-followed-by-dose-intensive-adjuvant-chemotherapy-for-children-with-newly-diagnosed-high-risk-medulloblastoma-protocol-pog-9631-a-report-from-the-children-s-oncology-group
#3
Adam J Esbenshade, Mehmet Kocak, Linda Hershon, Pierre Rousseau, Jean-Claude Decarie, Susan Shaw, Peter Burger, Henry S Friedman, Amar Gajjar, Albert Moghrabi
BACKGROUND: Children with high-risk medulloblastoma historically have had a poor prognosis. The Children's Oncology Group completed a Phase II study using oral etoposide given with radiotherapy followed by intensive chemotherapy. PROCEDURE: Patients enrolled in the study had high-risk disease defined as ≥1.5 cm(2) of residual disease postsurgery or definite evidence of central nervous metastasis. All patients underwent surgery followed by radiotherapy. During radiation, the patients received oral etoposide (21 days on, 7 off) at an initial dose of 50 mg/m(2) per day (treatment 1), which was reduced to 35 mg/m(2) per day (treatment 2) due to toxicity...
December 21, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27981412/survival-in-pediatric-medulloblastoma-a-population-based-observational-study-to-improve-prognostication
#4
Alexander G Weil, Anthony C Wang, Harrison J Westwick, George M Ibrahim, Rojine T Ariani, Louis Crevier, Sebastien Perreault, Tom Davidson, Chi-Hong Tseng, Aria Fallah
Medulloblastoma is the most common form of brain malignancy of childhood. The mainstay of epidemiological data regarding childhood medulloblastoma is derived from case series, hence population-based studies are warranted to improve the accuracy of survival estimates. To utilize a big-data approach to update survival estimates in a contemporary cohort of children with medulloblastoma. We performed a population-based retrospective observational cohort study utilizing the Surveillance, Epidemiology, and End Results Program database that captures all children, less than 20 years of age, between 1973 and 2012 in 18 geographical regions representing 28% of the US population...
December 15, 2016: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/27917595/societal-preferences-in-the-treatment-of-pediatric-medulloblastoma-balancing-risk-of-death-and-quality-of-life
#5
Amir Khakban, Tima Mohammadi, Larry D Lynd, Don Mabbott, Eric Bouffet, Louise Gastonguay, Zafar Zafari, David Malkin, Michael Taylor, Carlo A Marra
PURPOSE: Medulloblastoma is the most prevalent childhood brain cancer. Children with medulloblastoma typically receive a combination of surgery, radiation, and chemotherapy. The survival rate is high but survivors often have sequelae from radiotherapy of the entire developing brain and spinal cord. Ongoing genetic studies have suggested that decreasing the dose of radiation might be possible among children with favorable molecular variants; however, this may result in an increased disease recurrence...
December 4, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27889018/posterior-fossa-tumors
#6
REVIEW
Lara A Brandão, Tina Young Poussaint
Pediatric brain tumors are the leading cause of death from solid tumors in childhood. The most common posterior fossa tumors in children are medulloblastoma, atypical teratoid/rhabdoid tumor, cerebellar pilocytic astrocytoma, ependymoma, and brainstem glioma. Location, and imaging findings on computed tomography (CT) and conventional MR (cMR) imaging may provide important clues to the most likely diagnosis. Moreover, information obtained from advanced MR imaging techniques increase diagnostic confidence and help distinguish between different histologic tumor types...
February 2017: Neuroimaging Clinics of North America
https://www.readbyqxmd.com/read/27863192/treatment-of-children-and-adolescents-with-metastatic-medulloblastoma-and-prognostic-relevance-of-clinical-and-biologic-parameters
#7
André O von Bueren, Rolf-Dieter Kortmann, Katja von Hoff, Carsten Friedrich, Martin Mynarek, Klaus Müller, Tobias Goschzik, Anja Zur Mühlen, Nicolas Gerber, Monika Warmuth-Metz, Niels Soerensen, Frank Deinlein, Martin Benesch, Isabella Zwiener, Robert Kwiecien, Andreas Faldum, Udo Bode, Gudrun Fleischhack, Volker Hovestadt, Marcel Kool, David Jones, Paul Northcott, Joachim Kuehl, Stefan Pfister, Torsten Pietsch, Stefan Rutkowski
Purpose To assess an intensified treatment in the context of clinical and biologic risk factors in metastatic medulloblastoma. Patients and Methods Patients (4 to 21 years old, diagnosed between 2001 and 2007) received induction chemotherapy, dose-escalated hyperfractionated craniospinal radiotherapy, and maintenance chemotherapy. Subgroup status and other biologic parameters were assessed. Results In 123 eligible patients (median age, 8.2 years old; median follow-up, 5.38 years), 5-year event-free survival (EFS) and overall survival (OS) were 62% (95% CI, 52 to 72) and 74% (95% CI, 66 to 82), respectively...
December 2016: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/27834469/the-expression-of-fat1-is-associated-with-overall-survival-in-children-with-medulloblastoma
#8
Jianzhong Yu, Hao Li
PURPOSE: The FAT1 gene is involved in some cancers; however, its role in medulloblastoma is less clear. This study investigated the effects of FAT1 expression on the prognosis of medulloblastoma patients. METHODS: Whole exome sequencing was undertaken in 40 medulloblastoma patient samples. FAT1 mRNA and protein expression levels in normal and brain tumor tissues were determined by fluorescence quantitative PCR and immunohistochemistry, respectively. The association of FAT1 expression with overall survival (OS) was examined by Kaplan-Meier curve analysis with a log-rank test...
November 8, 2016: Tumori
https://www.readbyqxmd.com/read/27830395/glutamine-for-the-treatment-of-vincristine-induced-neuropathy-in-children-and-adolescents-with-cancer
#9
Stephen Sands, Elena J Ladas, Kara M Kelly, Michael Weiner, Meiko Lin, Deborah Hughes Ndao, Amie Dave, Linda T Vahdat, Julia Glade Bender
BACKGROUND: Vincristine is an integral treatment component of many childhood tumors with potentially dose-limiting sensory and/or motor neuropathy. Results from a pilot study on the incidence of vincristine-induced peripheral neuropathy (VIPN) as well as the efficacy and safety of glutamine in reducing signs and symptoms of VIPN in children with cancer are presented. METHODS: Fifty-six patients between the ages of 5-21 with newly diagnosed leukemia, lymphoma, extracranial solid tumor or medulloblastoma and expected to receive a minimum cumulative dose of 6 mg/m(2) of vincristine over a 30-week period were eligible...
November 9, 2016: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
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/27787875/posterior-fossa-syndrome-review-of-the-behavioral-and-emotional-aspects-in-pediatric-cancer-patients
#11
REVIEW
Jane C Lanier, Annah N Abrams
Medulloblastoma, the most common malignant brain tumor of childhood, occurs in the posterior fossa, the part of the intracranial cavity that contains the brainstem and the cerebellum. The cerebellum is involved in many complex aspects of human behavior and function, and when it is disrupted or insulted, this can lead to significant sequelae in children with posterior fossa tumors. A constellation of impairing and distressing symptoms, including mutism, ataxia/hypotonia, and emotional lability, develops in approximately 25% of children after the surgical resection of posterior fossa tumors...
October 27, 2016: Cancer
https://www.readbyqxmd.com/read/27775567/a-proteogenomic-approach-to-understanding-myc-function-in-metastatic-medulloblastoma-tumors
#12
REVIEW
Jerome A Staal, Yanxin Pei, Brian R Rood
Brain tumors are the leading cause of cancer-related deaths in children, and medulloblastoma is the most prevalent malignant childhood/pediatric brain tumor. Providing effective treatment for these cancers, with minimal damage to the still-developing brain, remains one of the greatest challenges faced by clinicians. Understanding the diverse events driving tumor formation, maintenance, progression, and recurrence is necessary for identifying novel targeted therapeutics and improving survival of patients with this disease...
October 19, 2016: International Journal of Molecular Sciences
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
#13
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/27764132/can-we-spare-the-pancreas-and-other-abdominal-organs-at-risk-a-comparison-of-conformal-radiotherapy-helical-tomotherapy-and-proton-beam-therapy-in-pediatric-irradiation
#14
Emmanuel Jouglar, Antoine Wagner, Grégory Delpon, Loïc Campion, Philippe Meingan, Valérie Bernier, Charlotte Demoor-Goldschmidt, Marc-André Mahé, Thomas Lacornerie, Stéphane Supiot
OBJECTIVES: Late abdominal irradiation toxicity during childhood included renal damage, hepatic toxicity and secondary diabetes mellitus. We compared the potential of conformal radiotherapy (CRT), helical tomotherapy (HT) and proton beam therapy (PBT) to spare the abdominal organs at risk (pancreas, kidneys and liver- OAR) in children undergoing abdominal irradiation. METHODS: We selected children with abdominal tumors who received more than 10 Gy to the abdomen...
2016: PloS One
https://www.readbyqxmd.com/read/27757491/-tumors-of-the-posterior-cranial-fossa
#15
P Papanagiotou, M Politi
Various types of brain tumor can occur in the region of the posterior fossa. Brain metastases in adults are the most common malignancies at this localization. Ependymomas, medulloblastomas and pilocytic astrocytomas occur mostly in children and only rarely in adults. Other tumors that occur in the posterior fossa are meningiomas, schwannomas, hemangioblastomas, brain stem gliomas and epidermoid tumors. Due to the fact that the various tumors of the posterior fossa have different treatment approaches and prognoses, an accurate and specific diagnosis is mandatory...
October 18, 2016: Der Radiologe
https://www.readbyqxmd.com/read/27695363/atypical-teratoid-rhabdoid-tumors-challenges-and-search-for-solutions
#16
REVIEW
Ahitagni Biswas, Lakhan Kashyap, Aanchal Kakkar, Chitra Sarkar, Pramod Kumar Julka
Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant embryonal central nervous system tumor commonly affecting children <3 years of age. It roughly constitutes 1%-2% of all pediatric central nervous system tumors. Recent data show that it is the most common malignant central nervous system tumor in children <6 months of age. Management of this aggressive tumor is associated with a myriad of diagnostic and therapeutic challenges. On the basis of radiology and histopathology alone, distinction of AT/RT from medulloblastoma or primitive neuroectodermal tumor is difficult, and hence this tumor has been commonly misdiagnosed as primitive neuroectodermal tumor for decades...
2016: Cancer Management and Research
https://www.readbyqxmd.com/read/27675855/adjuvant-radiation-therapy-patterns-and-survival-implications-for-medulloblastoma-in-young-children
#17
B H Kann, H S M Park, N H Lester-Coll, D N Yeboa, V Benitez, R S Bindra, A J Khan, A M Marks, K B Roberts
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/27633003/mps1-kinase-as-a-potential-therapeutic-target-in-medulloblastoma
#18
Irina Alimova, June Ng, Peter Harris, Diane Birks, Andrew Donson, Michael D Taylor, Nicholas K Foreman, Sujatha Venkataraman, Rajeev Vibhakar
Medulloblastoma is the most common type of malignant brain tumor that affects children. Although recent advances in chemotherapy and radiation have improved outcomes, high-risk patients perform poorly with significant morbidity. Gene expression profiling has revealed that monopolar spindle 1 (MPS1) (TTK1) is highly expressed in medulloblastoma patient samples compared to that noted in normal cerebellum. MPS1 is a key regulator of the spindle assembly checkpoint (SAC), a mitotic mechanism specifically required for proper chromosomal alignment and segregation...
November 2016: Oncology Reports
https://www.readbyqxmd.com/read/27607585/exploratory-analysis-of-ercc2-dna-methylation-in-survival-among-pediatric-medulloblastoma-patients
#19
Emilyn Banfield, Austin L Brown, Erin C Peckham, Surya P Rednam, Jeffrey Murray, M Fatih Okcu, Laura E Mitchell, Murali M Chintagumpala, Ching C Lau, Michael E Scheurer, Philip J Lupo
AIM: Medulloblastoma is the most frequent malignant pediatric brain tumor. While survival rates have improved due to multimodal treatment including cisplatin-based chemotherapy, there are few prognostic factors for adverse treatment outcomes. Notably, genes involved in the nucleotide excision repair pathway, including ERCC2, have been implicated in cisplatin sensitivity in other cancers. Therefore, this study evaluated the role of ERCC2 DNA methylation profiles on pediatric medulloblastoma survival...
October 2016: Cancer Epidemiology
https://www.readbyqxmd.com/read/27588198/medulloblastoma-in-an-elderly-patient-a-case-report-and-literature-review
#20
Bo Liang, Enshan Feng, Qinghe Wang, Yang Cao, Peiliang Li, Yanbin Li, Jidian Sun, Fang Wang
Medulloblastoma is the most common central nervous system malignancy in children; however, it is significantly less common among adults, particularly elderly individuals. We herein report an unusual case of a 72-year-old woman who presented with progressive dizziness. Magnetic resonance imaging revealed a heterogeneous solid mass located near the right tentorium of the cerebellum. The patient underwent surgical removal of the tumor and microscopic examination revealed histological and immunohistochemical characteristics consistent with a diagnosis of medulloblastoma...
September 2016: Molecular and Clinical Oncology
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