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Pontine Glioma


Presentation

  • At presentation, DIPG blood volume was 60 27 % of that measured for normal cerebellum. Citrate, which is not detectable in normal brain tissue, was present in DIPG at concentrations of 3.81 1.44 mmol/kg tissue.[ncbi.nlm.nih.gov]
  • Children with pontine glioma usually present classically with ataxia, motor deficits and cranial nerve palsies. The pons has generally not been regarded as a structure that mediates complex affective behaviour.[ncbi.nlm.nih.gov]
  • We present a case of inadvertent high-dose therapy with temozolomide in a child with recurrent diffuse intrinsic pontine glioma followed by a rapid clinical response.[ncbi.nlm.nih.gov]
  • The authors summarize the principles of nuclear magnetic resonance imaging and present a case report illustrating its particular application to the demonstration of a pontine glioma.[ncbi.nlm.nih.gov]
  • Diffuse intrinsic pontine gliomas are diagnosed by their clinical signs and symptoms on presentation and their appearance on imaging.[ispn.guide]
Death in Childhood
  • Numbers Brain tumours are the leading cause of death from childhood cancer. We estimate that approximately 300 children are diagnosed each year in the US with DIPG (interpolating this to the world population will lead to a few thousand globally).[lylansoulifoundation.org]
Plethora
  • A plethora of studies have investigated the immune system of patients with cancer and how it is prone to immunosuppression, due in part to the decrease in lymphocyte proliferation and cytotoxic activity.[ncbi.nlm.nih.gov]
Hoarseness
  • This 12-year-old boy presented with a 4-month history of hoarseness, dysphagia, and a progressively ataxic gait.[ncbi.nlm.nih.gov]
Aspiration
  • Barium swallow evaluation prior to transfer revealed laryngeal penetration with silent aspiration and absent cough reflex.[casereports.bmj.com]
Stridor
  • On physical examination the infant had stridor, increased oral secretions, axial hypotonia, right esotropia and facial weakness.[casereports.bmj.com]
Sore Mouth
  • Sore mouth (mucositis) Some of the chemotherapy drugs make the lining of the mouth and throat very sore and ulcerated. We will give your child painkillers for this, and explain how to care for your child’s mouth during treatment.[royalmarsden.nhs.uk]
Vertigo
  • Six months later, he presented ataxia syndrome with lateropulsion, star gait, dysarthria, aphasia, vertigo, and altered mental state. MRI showed an increase in the size of the tumor to 4.7 x 3.9 x 4.1 cm.[elsevier.es]
  • Still, she continued to participate in her freshman classes, often turning in handwritten homework as use of a computer screen triggered agonizing headaches and vertigo.[acco.org]
Long Tract Signs
  • Although the exact presentation will vary according to location and size of the tumor, in general patients will exhibit a combination of 4 : ataxia cranial nerve palsies long tract signs hydrocephalus The duration of symptoms is usually much shorter in[radiopaedia.org]
  • Pontine and medullary DMG present insidiously with brainstem signs (cranial nerve deficits, long tract signs) and progress inexorably, usually causing death in 2 years.[neuropathology-web.org]
Hand Tremor
  • DDx: Midbrain tegmental glioma Rhombencephalitis Non-neoplastic inflammatory cellular infiltrates Rx: Radiation therapy chemotherapy A 4-year-old girl with new-onset right hand tremor and several-day history of ataxic gait and headache View Case[ajnr.org]
Night Terrors
  • Calle developed night terrors. She began talking in her sleep for hours at a time. In September, Calle, a girl known for diving into situations with a playful attitude, did not want to attend kindergarten.[globalgenes.org]

Treatment

  • Treatment was well tolerated, and no DLTs were observed up to the maximum dose of 200 mg/m 2 . All patients experienced reduction of tumor-related symptoms. QoL tended to improve during treatment.[ncbi.nlm.nih.gov]
  • […] progressive DIPG to better assess treatment response.[ncbi.nlm.nih.gov]
  • The primary endpoint for first-line treatment was objective response rate (CR PR SD) according to the RECIST. This report concerns the outcome of this strategy as a whole.[ncbi.nlm.nih.gov]
  • In this section Treatment Effects of treatment In children, 80% of brain stem tumours are pontine gliomas.[royalmarsden.nhs.uk]
  • Nanotechnology may offer therapeutic options for the treatment of DIPGs. Multiple nanoparticle formulations are currently being investigated for the treatment of DIPGs.[eurekaselect.com]

Prognosis

  • […] of Pontine Glioma poor prognosis prognosis is tried to improve prognosis with therapy-optimising studies and preospective studies[hirntumorhilfe.org]
  • If you would like to know what the average prognosis is for DIPGs, please visit our prognosis page .[thebraintumourcharity.org]
  • Due to the poor prognosis of diffuse intrinsic pontine gliomas, the limited therapy options, the relevant portion of EGFR expression and the unexpected good response to the therapy with OSAG 101 in the phase II study, a phase III study was planned in[clinicaltrials.gov]
  • The prognosis and do-not-resuscitate orders are discussed upfront with patients' parents.[ncbi.nlm.nih.gov]

Epidemiology

  • The book provides basic researchers and clinicians with a contemporary review of the epidemiology, diagnosis, and treatment of glioblastoma multiforme, and also imparts to experienced investigators outside of the field sufficient background to apply their[books.google.es]
  • […] part of brain stem) in children and adolescents often pilocytic astrocytoma (Who grade I) in adults often diffuse (WHO grade II) or anaplastic (WHO grade III) astrocytoma classified as WHO-grade IV because they are difficult to treat due to locaisation Epidemiology[hirntumorhilfe.org]
  • The journal welcomes submissions concerning clinical trials, epidemiology, molecular and cellular biology, and genetics.[cancercommun.biomedcentral.com]
  • From Wikibooks, open books for an open world Jump to navigation Jump to search Epidemiology [ edit ] 10-20% CNS tumors in children 5% CNS tumors in adults Subtypes [ edit ] Diffuse brainstem glioma [ edit ] Usually located in the pons.[en.wikibooks.org]
Sex distribution
Age distribution

Pathophysiology

  • Possible pathophysiological mechanisms resulting in this unusual clinical presentation are discussed.[ncbi.nlm.nih.gov]
  • Title: Diffuse Intrinsic Pontine Glioma: New Pathophysiological Insights and Emerging Therapeutic Targets VOLUME: 15 ISSUE: 1 Author(s): Tessa B.[eurekaselect.com]
  • Yujie Tang & Yulun Huang Present addresses: Key Laboratory of Cell Differentiation and Apoptosis of National Ministry of Education, Department of Pathophysiology, Shanghai Jiao Tong University School of Medicine, Shanghai, China (Y.T.) and Department[nature.com]
  • Medulloblastoma; Diffuse Intrinsic Pontine Glioma; Primary CNS Tumor Drug: Indoximod; Drug: Temozolomide; Radiation: Conformal Radiation; Drug: Cyclophosphamide; Drug: Etoposide NewLink Genetics Corporation December 2019 An Exploratory Study of Biologic and Pathophysiologic[dipgregistry.org]

Prevention

  • The BBB is arguably the most formidable barrier for drug delivery to the brain and to brain tumors as it prevents drugs and drug delivery systems from reaching the pathological site.[era-learn.eu]
  • Terrance Johns Preventing resistance to targeted therapies Dr Lee Wong Investigating tumour initiating events Working on links with other diseases Dr Chris Jones and his team at the ICR have previously found a common genetic fault in the DNA of some children[thebraintumourcharity.org]
  • Prevention - Diffuse Intrinsic Pontine Glioma Not supplied. Diagnosis - Diffuse Intrinsic Pontine Glioma Diffuse pontine gliomas are located in the brainstem, at the base of the brain. They are usually diagnosed in children aged 5 to 10.[checkorphan.org]
  • However, with diseases such as brain tumors, the BBB can also prevent diagnostic and therapeutic agents from reaching their target.[en.wikipedia.org]
  • Hospital for Sick Children Health A-Z Search a complete list of child health articles expand_more View All Drug A-Z Search a list of articles about medications expand_more View All Learning Hubs Browse a complete list of content groups Healthy Living & Prevention[aboutkidshealth.ca]

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