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Auteur : Grafiati

Publié le 22 juin 2024

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1

Klimo, Paul, et Nir Shimony. Ependymomas. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190696696.003.0026.

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Pediatric posterior fossa tumors are usually ependymoma, pilocytic astrocytoma, or medulloblastoma. Ependymoma appears well-demarcated with heterogeneous enhancement on magnetic resonance imaging (MRI). Full neural axis MRI is indicated to assess for metastatic disease. Management is typically surgical resection of the tumor, with consideration for cerebrospinal fluid diversion if patients present with severe hydrocephalus. Extent of resection of the tumor is the most important factor in predicting recurrence and overall survival, and gross total resection is ideal. Infratentorial ependymomas have 2 molecular subtypes, which has implications for responsiveness to adjuvant therapy and prognosis. Infratentorial ependymomas are biologically different from supratentorial ependymomas. Postoperative radiation improves local control.

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2

Fowler, Raquel. Ependymomas : Prognostic Factors, Treatment Strategies and Clinical Outcomes. Nova Science Publishers, Incorporated, 2016.

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3

Theeler, Brett J., et Mark R. Gilbert. Primary Central Nervous System Tumors. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0129.

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Ependymomas are rare primary central nervous system (CNS) tumors that are thought to arise from ependymal cells lining the ventricular system located throughout the CNS. Ependymomas occur in all age groups but are more common in the pediatric population. Ependymomas typically present as mass lesions within the ventricular system, brain or spinal cord parenchyma. As with most central nervous system tumors, pathologic evaluation is required for definitive diagnosis. Ependymomas are typically treated with a combination of surgery and radiotherapy although this varies depending on tumor location, tumor grade, patient age, extent of tumor resection, and other pretreatment factors. Recent molecular studies demonstrate molecularly defined tumor heterogeneity that appears to have a region-specific pattern. Translating the emerging molecular profiles of ependymomas into improved treatment strategies is the primary goal of ongoing research efforts.

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4

Gilbert, Mark R., et Roberta Rudà. Ependymal tumours. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199651870.003.0005.

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Ependymomas are uncommon central nervous system cancers that can arise in the supratentorial, infratentorial, or spinal cord region. Recently, there have been several seminal findings regarding the molecular profiles of ependymomas that have led to marked changes in the classification of this disease. In addition to the World Health Organization grading system that designates ependymomas based on histological appearance into grade I, II, or III, a new molecular classification with distinct entities within the three anatomical regions provides additional subtyping that has prognostic significance and may ultimately provide therapeutic targets. Ependymomas are typically treated with maximum safe tumour resection. Grade III tumours always require radiation treatment even with extensive resection. Radiation is also often administered to patients with grade II ependymomas. Grade I tumours typically receive radiation if there is extensive residual disease, but complete resection may be curative. Local radiation is optimal unless there is imaging or cytological evidence of dissemination in the cerebrospinal fluid. Chemotherapy is less well established although recent molecular findings may lead to subtype specific treatments.

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5

Elwell, Christine, et Kufre Sampson. Neurological tumours. Sous la direction de Patrick Davey et David Sprigings. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199568741.003.0237.

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Neurological tumours are categorized by the WHO as follows: neuroepithelial tumours (gliomas, oligodendrogliomas, ependymomas, pineal parenchymal tumours, medulloblastoma, neuronal and neuroglial tumours); cranial and paraspinal nerve tumours (schwannoma, neurofibromas); meningeal tumours (meningiomas); lymphomas; germ cell tumours (germinoma, teratoma); sellar region tumours (cranipharyngioma); and metastases. The tumours are classified according to grade. The WHO histological grading scheme used for astrocytomas is based on mitoses, nuclear pleomorphism, necrosis, and endothelial proliferation. WHO Grade I and Grade II tumours are low-grade tumours, and WHO Grade III and Grade IV tumours are high-grade tumours.

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6

Haranhalli, Neil, et Jerome J. Graber. Pineal Region Neoplasms. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0131.

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Pineal region tumors include a diverse array of neoplasms arising from various components of the pineal gland, including germ cell tumors, germinomas, teratomas, pineocytomas, pineoblastomas, and tumors derived from glial tissues including gliomas, astrocytomas, oligodendrogliomas, and ependymomas. Benign lesions of the pineal gland can include pineal cysts, calcifications and meningiomas. Metastatic tumors can also be found in the pineal region. Numerous infectious and inflammatory conditions can mimic pineal tumors. Most patients present with symptoms of hydrocephalus or Parinaud’s syndrome. Diagnosis often requires biopsy, though some germinomas may be diagnosed based solely on serum and cerebrospinal fluid biomarkers.

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7

Hayat, M. A. Tumors of the Central Nervous System, Volume 9 : Lymphoma, Supratentorial Tumors, Glioneuronal Tumors, Gangliogliomas, Neuroblastoma in Adults, Astrocytomas, Ependymomas, Hemangiomas, and Craniopharyngiomas. Springer Netherlands, 2016.

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8

Hayat, M. A. Tumors of the Central Nervous System, Volume 9 : Lymphoma, Supratentorial Tumors, Glioneuronal Tumors, Gangliogliomas, Neuroblastoma in Adults, Astrocytomas, Ependymomas, Hemangiomas, and Craniopharyngiomas. Springer London, Limited, 2012.

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9

Taylor, Jennie W., et Scott R. Plotkin. Familial CNS Tumor Syndromes. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199937837.003.0135.

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Neurofibromatosis type 2 (NF2) is a genetic disorder caused by constitutional mutations in the NF2 tumor-suppressor gene. Bilateral vestibular schwannomas are the hallmark of the syndrome, though meningiomas, ependymomas, and other peripheral schwannomas are common. Inheritance is autosomal dominant and de novo mutations are found in about 50% of patients. Standard treatment for symptomatic tumors is surgery. Radiation therapy may be considered for progressive tumors that are not surgically accessible, but secondary cancers after radiation have been reported. Retrospective studies suggest that bevacizumab may be active for progressive vestibular schwannomas and trials of chemotherapy for NF2-related tumors are in progress. This chapter reviews the epidemiology, genetic features, clinical characteristics, and current treatments for patients with NF2.

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10

Ernestus, Ralf-Ingo. Klinik und Prognose der Intrakraniellen Ependymome. Lang AG International Academic Publishers, Peter, 1989.

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11

Rahman, Mohammad Mohsin. In-vivo and in-vitro characterization of a human ependymoma-derived cell line. 1985.

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12

Burnet, Neil G., Fiona Harris, Raj Jena, Kate E. Burton et Sarah J. Jefferies. Central nervous system tumours. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199696567.003.0016.

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Chapter 11 discusses central nervous system (CNS) tumours, including principles, planning volumes, dose distribution, high- and low-grade glioma, Ependymoma, Medulloblastoma, Germ cell tumours —germinoma and teratoma, Vestibular (acoustic) schwannoma, Pituitary tumours (including craniopharyngioma), Meningioma, and primary spinal cord tumours.

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13

Publications, ICON Health. The Official Parent's Sourcebook on Childhood Ependymoma : A Revised and Updated Directory for the Internet Age. ICON Health Publications, 2002.

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14

Hayat, M. A. Tumors of the Central Nervous System, Volume 8 : Astrocytoma, Medulloblastoma, Retinoblastoma, Chordoma, Craniopharyngioma, Oligodendroglioma, and Ependymoma. Springer Netherlands, 2016.

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15

Hayat, M. A. Tumors of the Central Nervous System, Volume 8 : Astrocytoma, Medulloblastoma, Retinoblastoma, Chordoma, Craniopharyngioma, Oligodendroglioma, and Ependymoma. Springer London, Limited, 2012.

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16

Carton, James. Neuropathology. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780198759584.003.0018.

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This chapter discusses neuropathology, including nervous system malformations, epilepsy, head injury, cerebral infarction, intracerebral haemorrhage, subarachnoid haemorrhage, meningitis, cerebral infections, multiple sclerosis, Guillain–Barré syndrome, myasthenia gravis, Alzheimer’s disease, vascular dementia, dementia with Lewy bodies, Parkinson’s disease, Huntington’s disease, motor neurone disease, Creutzfeldt–Jacob disease, astrocytomas (including glioblastoma), oligodendroglioma, ependymoma, meningioma, medulloblastoma, primary CNS lymphomas, and cerebral metastases.

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17

Cassidy, Jim, Donald Bissett, Roy A. J. Spence OBE, Miranda Payne, Gareth Morris-Stiff et Madhumita Bhattacharyya. Skin cancers. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780199689842.003.0023_update_001.

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Tumours of the central nervous system examines the epidemiology, aetiology, genetics and pathology of these heterogeneous tumours. Clinical presentation reflects the site of origin and rate of growth. Investigation usually comprises imaging (MRI superior to CT for most), and biopsy; requirement for additional staging depends on pathology. The treatment of low-grade gliomas may be delayed if small with few symptoms, otherwise surgery and/or radiotherapy. High grade gliomas may be managed with surgery, radiotherapy, and temozolomide chemotherapy in fit patients. Unfit patients should be offered supportive care only. Brief summaries are provided for management of ependymoma, pineal tumours, meningioma, germ-cell CNS tumours, pituitary tumours, CNS lymphoma, acoustic neuroma, medulloblastoma, and spinal cord tumours. Radiotherapy for primary CNS tumours is described along with its side effects, and chemotherapy for these diseases is reviewed. Brain metastases far outnumber primary brain tumours, with generally poor prognosis, but this relates both to the pathology and patient performance status. Appropriate treatment may include surgery, radiotherapy, and/or chemotherapy.

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18

Kleihues, Paul, Elisabeth Rushing et Hiroko Ohgaki. The 2016 revision of the WHO classification of tumours of the central nervous system. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199651870.003.0001.

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The revised fourth edition of the WHO classification of Tumours of the Central Nervous System, published in 2016, comprises several newly recognized tumour entities, and a significant restructuring of the classification, mainly based on genetic profiling. Glioblastomas are now classified into two major types. Isocitrate dehydrogenase (IDH)-wildtype glioblastoma (primary glioblastoma IDH-wildtype) develops rapidly de novo without a recognizable precursor lesion. IDH-mutant glioblastoma (secondary glioblastoma IDH-mutant) develops more slowly through malignant progression from diffuse or anaplastic astrocytoma. Medulloblastomas are now defined by combining histological patterns (classic, desmoplastic/nodular, extensive nodularity, anaplastic) and genetic hallmarks (WNT-activated; SHH-activated, TP53-mutant; SHH-activated, TP53-wildtype; non-WNT/non-SHH). Other newly recognized tumour entities include diffuse midline glioma, H3 K27M-mutant; ependymoma, RELA fusion-positive; and embryonal tumour with multilayered rosettes. The new classification is a significant step forward and will facilitate the development of novel targeted therapies of brain tumours.

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