Medulloblastoma |
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| Authors: | F. Bartlett R. Kortmann F. Saran |
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| Affiliation: | 1. Department of Radiotherapy, The Royal Marsden NHS Foundation Trust, Sutton, Surrey, UK;2. Department of Radiotherapy and Radiooncology, Universitatsklinikum Leipzig, Leipzig, Germany;3. Department of Neuro-Oncology and Paediatric Oncology, The Royal Marsden NHS Foundation Trust, Sutton, Surrey, UK;1. Wolfson Childhood Cancer Research Centre, Northern Institute for Cancer Research, Newcastle University, Newcastle upon Tyne, UK;2. Department of Applied Sciences, Northumbria University, Newcastle upon Tyne, UK;3. Department of Haematology and Oncology Department, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK;4. Neural Development Unit, UCL Institute of Child Health, London, UK;5. Institute of Translational Research, University of Liverpool, Liverpool, UK;6. Department of Neuropathology, Royal Victoria Infirmary, Newcastle University Teaching Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK;7. Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK;1. Harvard Radiation Oncology Program, Boston, Massachusetts;2. Harvard Medical School, Boston, Massachusetts;3. Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts;4. Department of Psychiatry, Massachusetts General Hospital, Boston, Massachusetts;1. Department of Medical Oncology, Bellaria-Maggiore Hospitals, Azienda USL – IRCCS Institute of Neurological Sciences, Bologna, Italy;2. Department of Biomedical and NeuroMotor Sciences (DiBiNeM), University, of Bologna, Section of Pathology, M. Malpighi, Bellaria Hospital, Bologna, Italy;3. Pathology Department, Verona Hospital, Verona, Italy;4. Department of Neuroradiology, Bellaria-Maggiore Hospitals, Azienda USL – IRCCS Institute of Neurological Sciences, Bologna, Italy;5. Department of Neurosurgery, Bellaria Hospital – IRCCS Institute of Neurological Sciences, Azienda USL, Bologna, Italy;6. Department of Radiotherapy Unit, CRO, Aviano, Italy;7. Department of Neuroscience and Neurosurgery, San Bortolo Hospital, Vicenza, Italy;8. Department of Neurosurgery I, Careggi University Hospital, Firenze, Italy;9. Section of Neurosurgery, Department of Neuroscience, University of Verona, Verona, Italy;10. Surgical Pathology & Cytopathology Unit, Department of Medicine (DIMED), University Hospital, Padova, Italy;11. Department of Medicine (DIMES) – Anatomic Pathology Unit, Bellaria Hospital, University of Bologna, Bologna, Italy;12. Department of Neuroradiology, Parma University Hospital, Parma, Italy;1. Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden;2. Department of Oncology–Pathology, Karolinska Institutet, Stockholm, Sweden;3. Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden;1. Gippsland Medical School, Northways Road, Monash University, Churchill, Victoria 3842, Australia;2. Department of Neurosurgery, The Royal Melbourne Hospital, Parkville, Victoria, Australia;3. Department of Surgery, University of Melbourne, Parkville, Victoria, Australia |
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| Abstract: | Medulloblastomas are primary malignant embryonal tumours of the central nervous system. They are the most common childhood central nervous system tumour, but are rare in the adult population. They arise infratentorially in the cerebellum or fourth ventricle and hence the most common presenting symptoms are those associated with raised intracranial pressure. Several histological subtypes have been described, although the classical and desmoplastic subtypes account for the majority. Recent advances in molecular biology and cytogenetics have led to an improved understanding of the genetic abnormalities and alterations in cell signalling pathways associated with medulloblastomas, including how these relate to patient outcome. The Modified Chang Staging System is still in use, but a number of other factors, including age, completeness of resection, histological subtype and genetic markers now contribute to treatment decisions and prognostication. Patients are currently classified as being either standard or high risk in order to stratify treatment. There has been an improvement in survival of all groups over the past 20 years. A multimodality approach is the cornerstone of treatment and recent trials have concentrated on ascertaining the most efficacious treatment combinations and timings for each patient group. Advances in surgical techniques have allowed a greater attainment of the two primary surgical goals: restoring normal cerebrospinal fluid (CSF) flow and maximal tumour resection. Radiotherapy to the craniospinal axis with a boost to the posterior fossa has been standard practice, but improvement in radiotherapy techniques and quality control has enabled optimisation of the trade-off between tumour control and normal tissue late toxicities. Combination chemotherapy is usually given adjuvantly, although it may be used to delay or avoid the use of radiotherapy in infants. In the future, the treatment of medulloblastoma will probably become increasingly individualised, based on patient-specific genetic features. Attention will be focussed not only on improving survival, but also on maintaining quality of life. |
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