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Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Leigh syndrome can have similar imaging appearances but occurs in young children and the mammillary bodies are spared. Pontine glioma are also more common in children. Furthermore, the supra- and infratentorial abnormalities in this case are not consistent with this diagnosis. Osmotic demyelination is possible in patients with chronic alcohol misuse due to rapid correction of hyponatraemia; however, the radiological features are more consistent with Wernicke encephalopathy.
Neurosurgical Techniques and Strategies
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Jonathan E. Martin, Ian F. Pollack, Robert F. Keating
Stereotactic biopsy provides a low-risk means of tissue sampling in deep-seated, eloquent areas. Technologic advances now allow for both frame-based biopsy and frameless stereotactic systems to be employed. In most locations, needle-based biopsies carry low risk of hemorrhage or permanent neurologic injury. This includes most locations in the diencephalon and cerebral hemispheres. Lesions in the pineal region are associated with elevated hemorrhage risk, with a mortality of up to 8%.28 Evolving understanding of tumor genomics has resulted in a revival of interest29 in the biopsy of brainstem lesions, to include diffuse pontine glioma.30 Risk of permanent neurologic deficit from brainstem biopsy is estimated to be 0.6%.30
Anti-EGFR nimotuzumab for DIPG in recurrent or children with high grade glioma: 10 years
Published in Cut Adeya Adella, Stem Cell Oncology, 2018
U. Bone, R. Cabanas, G. Saurez-Martinez, T. Crombet Ramos, P. Lorenzo-Luaces, M. Massimino, U. Bartels, E. Bouffet, F. Bach, D. Reuter, R.A. Ilyas, R. Ellerson, N. Iznaga-Escobar
Glioma is a rare but devastating cancer. Although temozolomide is currently the first-line standard of care for the treatment of glioma, temozolomide has shown minimal activity in high-grade glioma of childhood with no convincing evidence of its activity in diffuse intrinsic pontine glioma (DIPG) (Fangusaro, 2012). To date, there is no standard therapy to treat newly diagnosed DIPG or recurrent or relapse high-grade glioma in children and adolescents.
Re-irradiation for recurrent/progressive pediatric brain tumors: from radiobiology to clinical outcomes
Published in Expert Review of Anticancer Therapy, 2023
Mohamed S. Zaghloul, Alistair Hunter, Ayatullah G. Mostafa, Jeannette Parkes
Meaningful palliation and prolonged survival for a few months were achieved through re-irradiating patients with progressive diffuse intrinsic pontine glioma (DIPG) upon progression after initial focal radiotherapy with or without chemotherapy [2,48,49]. The interval between initial radiation and progression (>6 months) and toxicity with the first irradiation were the prognostic factors determining the length of the second PFS [50,51]. The re-irradiation dose ranged between 18 and 36 Gy, with no apparent statistical differences between dose levels. Many centers use re-reirradiation without reporting much toxicity [52]. In a retrospective study comparing 82 cases of progressive DIPG who received re-irradiation 20–26 Gy to 89 comparable patients of progressive DIPG who received the best supportive care, the median OS was 6.9 and 2.4 months, respectively (p = 0.001). Re-irradiation offered 4.5 months’ prolongation of OS. The authors found that the only significant prognostic factor was a clinical improvement after re-irradiation (p = 0.001). On the other hand, the MR improvement had only a trend of statistical significance (p = 0.07) [53]. On the other hand, SIOP-E showed 31 children with progressive DIPG with a re-irradiation dose of ≥20 Gy provides a neurological improvement of symptoms in more than 60% of patients. However, there was no improvement in survival between responding and non-responding patients [54].
Immunotherapy approaches for the treatment of diffuse midline gliomas
Published in OncoImmunology, 2022
Joshua D. Bernstock, Samantha E. Hoffman, Ari D. Kappel, Pablo A. Valdes, Walid Ibn Essayed, Neil V. Klinger, Kyung-Don Kang, Stacie K. Totsch, Hannah E. Olsen, Charles W. Schlappi, Katharina Filipski, Florian A. Gessler, Lissa Baird, Mariella G. Filbin, Rintaro Hashizume, Oren J. Becher, Gregory K. Friedman
Central nervous system (CNS) tumors are the most common solid malignancy in children and are the primary cause of pediatric cancer-associated mortality.1 Pediatric high-grade gliomas (pHGGs) are aggressive neoplasms that originate from glial lineages in the developing CNS. While many types of pHGGs commonly occur in the cerebral hemispheres, including pediatric glioblastoma (GBM) and anaplastic astrocytomas, some of the most lethal subtypes arise in the thalamus, spinal cord, and/or brainstem, where they are subclassified as diffuse midline gliomas (DMG). Up to 80% of DMGs arise in the pons, where they are also referred to as diffuse intrinsic pontine glioma (DIPG).2,3 Pathogenetically, the 2021 World Health Organization (WHO) classification of CNS tumors distinguishes isocitrate dehydrogenase (IDH)- and histone 3 (H3)-wildtype pHGGs, which may sometimes arise in midline structures, from H3 lysine 27 (K27)-altered DMG.4 Irrespective of the location and pathogenetic molecular alteration, H3K27-altered DMGs are classified as WHO grade 4 tumors and bear dismal prognoses.4
Targeting the DNA damage response in pediatric malignancies
Published in Expert Review of Anticancer Therapy, 2022
Jenna M Gedminas, Theodore W Laetsch
PARP inhibitors have also been evaluated in pediatric patients with recurrent CNS tumors by the Pediatric Brain Tumor Consortium. In a phase I trial of veliparib plus temozolomide no objective responses were seen but 4/12 patients treated at the RP2D had stable disease for greater than 6 months and the combination was well tolerated [108]. Following that study, a phase I/II study of veliparib plus temozolomide and radiation was completed in patients with newly diagnosed diffuse pontine glioma. Patients were given veliparib Monday through Friday twice daily during radiation and then, after a 4-week rest period, received veliparib twice daily and temozolomide daily for 5 days every 28 days. The study was discontinued early due to a lack of survival benefit over historical controls with 1-year overall survivals of 37.2% vs 45.6%, respectively [109].