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posterior fossa tumours

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https://www.readbyqxmd.com/read/28512124/use-of-propranolol-in-a-remote-region-of-rural-guatemala-to-treat-a-large-facial-infantile-haemangioma
#1
Vera Goldberg, Boris Martinez, Katia Cnop, Peter Rohloff
We present a female infant with a right-sided facial and neck haemangioma, from a remote, resource-poor community in rural Guatemala. She received first-line treatment, propranolol, with marked reduction in tumour size and erythema. Treatment was stopped after 35 weeks due to recurrent diarrhoea and sustained weight loss. Propranolol can be used to safely treat infants with haemangiomas in remote, rural communities if there is adequate follow-up, education and communication. Periocular haemangiomas should be treated promptly to avoid visual impairment...
May 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28503272/von-hippel-lindau-disease
#2
Sna Muhamad Nor, J Haron
Von Hippel-Lindau (VHL) disease is a rare autosomal dominantly inherited multisystem disorder characterised by the development of a variety of benign and malignant tumours. We report a case of VHL disease that was inherited by a daughter from her father, who both presented at a young age with progressive headache and were found to have a posterior fossa haemangioblastoma (HB) on magnetic resonance imaging (MRI). Multiple benign pancreatic and renal cysts were also noted in both patients.
2017: Malaysian Family Physician
https://www.readbyqxmd.com/read/28432426/posterior-cranial-fossa-tumours-in-children-at-national-cancer-institute-sudan-a-single-institution-experience
#3
Moawia Mohammed Ali Elhassan, Haytham Hussein Mohammed Osman, Jeannette Parkes
BACKGROUND: Posterior cranial fossa tumours (PCF) comprise 54-70% of childhood brain tumours. The clinical profile and outcomes of these tumours differ in area of the world. The aim of this study is to describe clinical characteristics, treatment and outcome of posterior cranial fossa tumours in Sudan. MATERIAL AND METHODS: A retrospective study of children diagnosed with posterior cranial fossa tumours and treated between January 1998 and December 2015 at National Cancer Institute, Sudan...
April 21, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28423966/linear-energy-transfer-distributions-in-the-brainstem-depending-on-tumour-location-in-intensity-modulated-proton-therapy-of-paediatric-cancer
#4
Lars Fredrik Fjæra, Zuofeng Li, Kristian S Ytre-Hauge, Ludvig P Muren, Daniel J Indelicato, Yasmin Lassen-Ramshad, Grete May Engeseth, Marianne Brydøy, Andrea Mairani, Stella Flampouri, Olav Dahl, Camilla H Stokkevåg
BACKGROUND: For tumours near organs at risk, there is concern about unintended increase in biological dose from elevated linear energy transfer (LET) at the distal end of treatment fields. The objective of this study was therefore to investigate how different paediatric posterior fossa tumour locations impact LET and biological dose to the brainstem during intensity-modulated proton therapy (IMPT). MATERIAL AND METHODS: Multiple IMPT plans were generated for four different simulated tumour locations relative to the brainstem for a five-year-old male patient...
April 19, 2017: Acta Oncologica
https://www.readbyqxmd.com/read/28318880/highly-conformal-craniospinal-radiotherapy-techniques-can-underdose-the-cranial-clinical-target-volume-if-leptomeningeal-extension-through-skull-base-exit-foramina-is-not-contoured
#5
D J Noble, T Ajithkumar, J Lambert, I Gleeson, M V Williams, S J Jefferies
AIMS: Craniospinal irradiation (CSI) remains a crucial treatment for patients with medulloblastoma. There is uncertainty about how to manage meningeal surfaces and cerebrospinal fluid (CSF) that follows cranial nerves exiting skull base foramina. The purpose of this study was to assess plan quality and dose coverage of posterior cranial fossa foramina with both photon and proton therapy. MATERIALS AND METHODS: We analysed the radiotherapy plans of seven patients treated with CSI for medulloblastoma and primitive neuro-ectodermal tumours and three with ependymoma (total n = 10)...
March 15, 2017: Clinical Oncology: a Journal of the Royal College of Radiologists
https://www.readbyqxmd.com/read/28137902/lhermitte-duclos-disease-associated-to-cowden-syndrome-de-novo-diagnosis-and-management-of-these-extremely-rare-syndromes-in-a-patient
#6
Ivo Gama, Leonor Almeida
A 36-year-old woman, with history of cutaneous papilomatosis and thyroid carcinoma presented with headache, transitory visual blurring and nausea. Funduscopy showed papilloedema. MRI showed a tumour of the right cerebellar hemisphere with a striated, tigroid pattern, typical of Lhermitte-Duclos disease (LDD). Significant clinical and perimetric improvements were noted after surgery and the follow-up did not reveal recurrences of the tumour. LDD is an extremely rare differential diagnosis of posterior fossa tumours...
January 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28096224/supra-and-infratentorial-ectopic-schwannoma-mimicking-a-meningioma
#7
Pedro Ribeiro da Cunha, José Luís Alves, Armando Rocha
We report the case of a supra and infratentorial ectopic schwannoma, mimicking a meningioma, that was surgically resected on a 75-year-old man, with a history of progressive visual loss and headaches. MRI was in favour of an occipital, cystic, tentorial-based meningioma extending to the posterior fossa, with signs of intratumoral haemorrhage. This is a unique case because of the rare type of lesion, in a very atypical location, completely unexpected by the preoperative imaging analysis, and to the best of our knowledge it is the oldest patient reported in the literature with this kind of tumour...
January 17, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/27942479/posterior-fossa-syndrome-a-narrative-review
#8
REVIEW
Salima S Wahab, Samantha Hettige, Kshtij Mankad, Kristian Aquilina
Posterior fossa syndrome (PFS), or cerebellar mutism syndrome (CMS), is a collection of neurological symptoms that occur following surgical resection of a posterior fossa tumour, and is characterised by either a reduction or an absence of speech. Some authors suggest that CM is only one symptom of the CMS complex that also includes ataxia, hypotonia and irritability as well as cranial nerve deficits, neurobehavioral changes and urinary retention or incontinence. It is seen almost exclusively in children. In 1985 Rekate et al...
October 2016: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/27942473/post-operative-pediatric-cerebellar-mutism-syndrome-and-its-association-with-hypertrophic-olivary-degeneration
#9
Shivaram Avula, Michaela Spiteri, Ram Kumar, Emma Lewis, Srikrishna Harave, David Windridge, Chan Ong, Barry Pizer
BACKGROUND: The dentato-thalamo-cortical (DTC) pathway is recognized as the anatomical substrate for postoperative pediatric cerebellar mutism (POPCMS), a well-recognized complication affecting up to 31% of children undergoing posterior fossa brain tumour resection. The proximal structures of the DTC pathway also form a segment of the Guillain and Mollaret triangle, a neural network which when disrupted causes hypertrophic olivary degeneration (HOD) of the inferior olivary nucleus (ION)...
October 2016: Quantitative Imaging in Medicine and Surgery
https://www.readbyqxmd.com/read/27866097/current-therapy-and-the-evolving-molecular-landscape-of-paediatric-ependymoma
#10
REVIEW
Soumen Khatua, Vijay Ramaswamy, Eric Bouffet
Ependymomas are the third commonest paediatric central nervous system (CNS) tumour, accounting for 6-12% of brain tumours in children. The management of these tumours has seen considerable changes over the last two decades, leading to a significant improvement in outcomes. However, despite advances in neurosurgical, neuroimaging and postoperative adjuvant therapy, management of these tumours remain challenging, and recurrence occurs in over 50% of cases, particularly when complete resection is not achieved prior to conformal radiotherapy...
January 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/27810194/-ectopic-craniopharyngioma-and-gardner-s-syndrome-case-report-and-literature-review
#11
Juan Antonio Álvarez Salgado, Francisco González-Llanos Fernández de Mesa, Jorge Javier Villaseñor Ledezma, Maria de Los Angeles Cañizares Méndez, Igor Paredes Sansinenea, Angel Rodríguez de Lope-Llorca, Manuela Mollejo Villanueva
INTRODUCTION: Craniopharyngioma accounts for around 3% of all primary tumours of the central nervous system. It is usually located in the suprasellar region, although it may also have an ectopic location. CASE REPORT: The case is presented on 29 year-old male who underwent surgery for a jaw osteoma when he was 19 years old and was subsequently diagnosed with Gardner's syndrome. He was admitted in our Hospital with right facial paresis and diplopia of one day onset...
October 31, 2016: Neurocirugía
https://www.readbyqxmd.com/read/27717899/postoperative-complications-after-craniotomy-for-brain-tumor-surgery
#12
Laurent Lonjaret, Marine Guyonnet, Emilie Berard, Marc Vironneau, Françoise Peres, Sandrine Sacrista, Anne Ferrier, Véronique Ramonda, Corine Vuillaume, Franck-Emmanuel Roux, Olivier Fourcade, Thomas Geeraerts
INTRODUCTION: After elective craniotomy for brain tumour surgery, patients are usually admitted to an intensive care unit (ICU) for monitoring. Our goal was to evaluate the incidence and timing of neurologic and non-neurologic postoperative complications after brain tumour surgery, to determine factors associated with neurologic events and to evaluate the timing and causes of ICU readmission. PATIENTS AND METHODS: This prospective, observational and analytic study enrolled 188 patients admitted to the ICU after brain tumour surgery...
October 4, 2016: Anaesthesia, Critical Care & Pain Medicine
https://www.readbyqxmd.com/read/27619980/surgical-management-of-posterior-fossa-metastases
#13
Geraint J Sunderland, Michael D Jenkinson, Rasheed Zakaria
The diagnosis of brain metastases is associated with a poor prognosis reflecting uncontrolled primary disease that has spread to the relative sanctuary of the central nervous system. 20 % of brain metastases occur in the posterior fossa and are associated with significant morbidity. The risk of acute hydrocephalus and potential for sudden death means these metastases are often dealt with as emergency cases. This approach means a full pre-operative assessment and staging of underlying disease may be neglected and a proportion of patients undergo comparatively high risk surgery with little or no survival benefit...
December 2016: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/27585992/assessing-second-look-tumour-resectability-in-childhood-posterior-fossa-ependymoma-a-centralised-review-panel-and-staging-tool-for-future-studies
#14
Christopher P Millward, Conor Mallucci, Tim Jaspan, Donald Macarthur, Richard Heyward, Tim Cox, Kung Chong, Richard G Grundy
PURPOSE: To improve uniformity in radiological review/reporting and discussion of surgical resection status in the forthcoming SIOP Ependymoma II trial, a central review of imaging and resection status will be carried out prior to treatment stratification. We present a review of surgical decision-making from the UKCCSG/SIOP Ependymoma trial for very young children (<3 years) (Lancet Oncol 8:696-705, 2007) and propose a primary surgical staging system for residual disease that could be used for Ependymoma II...
November 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27569564/-syringomyelias-in-paediatrics-a-retrospective-study-of-25-cases
#15
I Martinez-Albaladejo, M Prochazkova, I Perez-Sebastian, B Bernardino-Cuesta, C Martinez-Ferrandez, O M Suarez-Traba, M Budke, J J Garcia-Penas
INTRODUCTION: Syringomyelia is defined as a cavity containing cerebrospinal fluid inside the spinal cord. AIM: To describe the clinical characteristics of a series of patients with syringomyelia, as well as its diagnosis and treatment. PATIENTS AND METHODS: We conducted a retrospective descriptive study by reviewing the medical records at our centre. RESULTS: We reviewed 25 patients diagnosed with syringomyelia. In five cases, the diagnosis was reached casually, and eight of them presented a previous severe pathology (tumour, bone or vascular)...
September 1, 2016: Revista de Neurologia
https://www.readbyqxmd.com/read/27556362/low-dose-actinomycin-d-treatment-re-establishes-the-tumoursuppressive-function-of-p53-in-rela-positive-ependymoma
#16
Theophilos Tzaridis, Till Milde, Kristian W Pajtler, Sebastian Bender, David T W Jones, Simone Müller, Andrea Wittmann, Magdalena Schlotter, Andreas E Kulozik, Peter Lichter, V Peter Collins, Olaf Witt, Marcel Kool, Andrey Korshunov, Stefan M Pfister, Hendrik Witt
Ependymomas in children can arise throughout all compartments of the central nervous system (CNS). Highly malignant paediatric ependymoma subtypes are Group A tumours of the posterior fossa (PF-EPN-A) and RELA-fusion positive (ST-EPN-RELA) tumours in the supratentorial compartment. It was repeatedly reported in smaller series that accumulation of p53 is frequently observed in ependymomas and that immunohistochemical staining correlates with poor clinical outcome, while TP53 mutations are rare. Our TP53 mutation analysis of 130 primary ependymomas identified a mutation rate of only 3%...
September 20, 2016: Oncotarget
https://www.readbyqxmd.com/read/27465421/rare-primary-pleomorphic-adenoma-in-posterior-fossa
#17
Liqun Yang, Leong Tan, Queenie Lau, Rumal Jayalath
BACKGROUND: Pleomorphic adenoma is a benign neoplastic tumor of the salivary gland. Salivary gland tumors in the intracranial cavity are generally restricted to the pituitary gland and sellar region. To our knowledge, there has been only 1 previous case report of a primary central nervous system pleomorphic adenoma outside of the sellar region. In that case report of a posterior fossa pleomorphic adenoma, typical myxochondroid stroma was not identified on histology, and its pathogenesis was not explored...
September 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27423645/relapse-patterns-and-outcome-after-relapse-in-standard-risk-medulloblastoma-a-report-from-the-hit-siop-pnet4-study
#18
Magnus Sabel, Gudrun Fleischhack, Stephan Tippelt, Göran Gustafsson, François Doz, Rolf Kortmann, Maura Massimino, Aurora Navajas, Katja von Hoff, Stefan Rutkowski, Monika Warmuth-Metz, Steven C Clifford, Torsten Pietsch, Barry Pizer, Birgitta Lannering
The HIT-SIOP-PNET4 randomised trial for standard risk medulloblastoma (MB) (2001-2006) included 338 patients and compared hyperfractionated and conventional radiotherapy. We here report the long-term outcome after a median follow up of 7.8 years, including detailed information on relapse and the treatment of relapse. Data were extracted from the HIT Group Relapsed MB database and by way of a specific case report form. The event-free and overall (OS) survival at 10 years were 76 ± 2 % and 78 ± 2 % respectively with no significant difference between the treatment arms...
September 2016: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/27417707/-astrocytoma-and-epilepsy-clinical-case
#19
Sergio Moreno-Jiménez, Karen Alejandra Miranda-Fernández, Mónica García Gutiérrez, Norma Vázquez-Estrada, Stephanie Müller-Grohmann, Fabiola Flores-Vázquez
BACKGROUND: Pilocytic astrocytoma is a rare tumour, usually occurring in paediatric ages, and mainly located in the posterior fossa. It can cause hydrocephalus and intracranial hypertension and, less frequently, seizures, or a focal neurological deficit. The main imaging study by magnetic resonance imaging, which shows a tumour with solid and cystic components without peri-lesional swelling. The election treatment is surgical, and the patient is considered cured if a total resection is accomplished...
July 11, 2016: Cirugia y Cirujanos
https://www.readbyqxmd.com/read/27375228/childhood-medulloblastoma
#20
REVIEW
Maura Massimino, Veronica Biassoni, Lorenza Gandola, Maria Luisa Garrè, Gemma Gatta, Felice Giangaspero, Geraldina Poggi, Stefan Rutkowski
Medulloblastoma accounts for 15-20% of childhood nervous system tumours. The risk of dying was reduced by 30% in the last twenty years. Patients are divided in risk strata according to post-surgical disease, dissemination, histology and some molecular features such as WNT subgroup and MYC status. Sixty to 70% of patients older than 3 years are assigned to the average-risk group. High-risk patients include those with disseminated and/or residual disease, large cell and/or anaplastic histotypes, MYC genes amplification...
September 2016: Critical Reviews in Oncology/hematology
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