Abstract
Tumors of the central nervous system (CNS) represent approximately 20% of all pediatric neoplasms. Of these, 4–6% are primary spinal cord tumors. Multiple primary cranio-spinal tumors are often associated with inherited syndromes including neurofibromatosis type 2 (NF2). The principal clinical feature of NF2 is the existence of vestibular schwannomas, meningiomas, and ependymomas; therefore, NF2 is also called MISME (multiple inherited schwannomas, meningiomas, and ependymomas) syndrome. Consequently, a multidisciplinary approach is needed for these patients, and diagnosis and treatment should be performed in an appropriate oncological center with a neurosurgeon, otolaryngologist, neurologist, geneticist, ophthalmologist, pathologist, radiologist, audiologist, and experienced nursing staff. Magnetic resonance imaging of the brain and spinal canal with contrast is the method of choice for diagnosis of CNS tumors. Surgical procedures are often necessary to establish a diagnosis, perform complete or incomplete excision of the tumor mass, and to reduce intracranial pressure. Radiotherapy and chemotherapy are adjuvant treatment options.
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Stachowicz-Stencel, T. (2012). Pediatric Multiple Primary Cranio-Spinal Tumors Associated with Neurofibromatosis Type 2: Combined Therapeutical Strategies. In: Hayat, M. (eds) Pediatric Cancer, Volume 3. Pediatric Cancer, vol 3. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-4528-5_24
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DOI: https://doi.org/10.1007/978-94-007-4528-5_24
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