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Hirntumoren

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Kompendium Internistische Onkologie

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Zusammenfassung

Im Kindesalter ist das Gehirn die häufigste Lokalisation solider Tumoren. Etwa die Hälfte der Fälle der Betroffenen hat ein Astrozytom niedriggradiger Malignität, das somit die häufigste von vielen, teils neu charakterisierten Entitäten ist. Der häufigste WHO-Grad-IV-Tumor ist das Medulloblastom, für das bislang die meisten Erkenntnisse vorliegen. Daher wird dieser Tumor ausführlicher dargestellt. Auf andere wichtige Hirntumoren beim Kind wird kurz vergleichend eingegangen. Neben der histologischen Diagnose wurden für die meisten Entitäten biologische Subgruppen identifiziert, deren Abgrenzung beim Medulloblastom bereits therapeutische Relevanz hat (z. B. WNT, SHH, Gruppe 3, Gruppe 4). Zudem gewinnt die Abklärung molekularer Parameter in Tumor und Keimbahn zunehmend an Bedeutung. Ebenso sind eine adäquate initiale Bildgebung und weitere Staging-Untersuchungen die Basis der postoperativen Versorgung und ggf. einer weiteren risikoadaptierten multimodalen Therapie (Radiotherapie, Chemotherapie).

Das Kapitel wurde in der 4. Auflage mitverfasst von Prof. Dr. med. Joachim Kühl, Universitätskinderklinik Würzburg.

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Literatur

  • Bailey CC, Gnekow A, Wellek S et al (1995) Prospective randomised trial of chemotherapy given before radiotherapy in childhood medulloblastoma. International Society of Pediatric Oncology (SIOP) and the German Society of Pediatric Oncology (GPO): SIOP II. Med Ped Oncol 25:166–178

    Article  CAS  Google Scholar 

  • Bloom HJG, Thorton H, Schweisguth O (1982) SIOP medulloblastoma and high grade ependymoma therapeutic clinical trail: preliminary results (1975–1981). In: Raybauld C, Clement R, Lebreuli G, Bernard JL (Hrsg) Pediatric oncology. Excerpta Medica, Amsterdam Oxford Pincerton, S 309–322

    Google Scholar 

  • Bueren AO von, Kortmann RD, von Hoff K et al (2016) Treatment of children and adolescents with metastatic medulloblastoma and prognostic relevance of clinical and biological parameters. J Clin Oncol., in press 34:4151–4160

    Google Scholar 

  • Calaminus G, Kortmann R, Worch J et al (2013) SIOP CNS GCT 96: final report of outcome of a prospective, multinational nonrandomized trial for children and adults with intracranial germinoma, comparing craniospinal irradiation alone with chemotherapy followed by focal primary site irradiation for patients with localized disease. Neuro Oncol 15(6):788–796

    Article  CAS  Google Scholar 

  • Caputy AJ, Mc Cullough DC, Manz HJ et al (1987) A review of the factors influencing the prognosis of medulloblastoma. The importance of cell differentiation. J Neurosurg 66:80–87

    Article  CAS  Google Scholar 

  • Chang CH, Housepian EM, Herbert C (1969) An operative staging system and a megavolt radiotherapeutic technique for cerebellar medulloblastoma. Radiology 93:1351–1359

    Article  CAS  Google Scholar 

  • Duffner PK, Horowitz ME, Krischer JP et al (1993) Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumor [see comments]. N Engl J Med 328(24):1725–1731

    Article  CAS  Google Scholar 

  • Gajjar A, Chintagumpala M, Ashley D et al (2006) Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicentre trial. Lancet Oncol 7:813–820

    Article  Google Scholar 

  • Gnekow AK, Falkenstein F, von Hornstein S et al (2012) Long-term follow-up of the multicenter, multidisciplinary treatment study HIT-LGG-1996 for low-grade glioma in children and adolescents of the German Speaking Society of Pediatric Oncology and Hematology. Neuro Oncol 10:1265–1284

    Article  Google Scholar 

  • Hoff K von, Hinkes B, Gerber NU et al (2009) Long-term outcome and clinical prognostic factors in children with medulloblastoma treated in the prospective randomised multicentre trial HIT'91. Eur J Cancer 45:1209–1217

    Google Scholar 

  • Jakacki RI, Burger PC, Zhou T et al (2012) Outcome of children with metastatic medulloblastoma treated with carboplatin during craniospinal radiotherapy: a Children's Oncology Group Phase I/II study. J Clin Oncol 30:2648–2653

    Article  CAS  Google Scholar 

  • Kortmann RD, Kuhl J, Timmermann B et al (2000) Postoperative neoadjuvant chemotherapy before radiotherapy compared to immediate radiotherapy followed by maintenance chemotherapy in the treatment of medulloblastoma in childhood: results of the German prospective randomized trial HIT ’91. Int J Radiat Oncol Biol Phys 46:269–279

    Article  CAS  Google Scholar 

  • Lannering B, Rutkowski S, Doz F et al (2012) Hyperfractionated versus conventional radiotherapy followed by chemotherapy in standard-risk medulloblastoma: results from the randomized multicenter HIT-SIOP PNET 4 trial. J Clin Oncol 30:3187–3193

    Article  Google Scholar 

  • Louis DN, Ohgaki H, Wiestler OD et al (2007) The 2007 WHO Classification of Tumours of the Central Nervous System. Acta Neuropathol 114(2):97–109

    Article  Google Scholar 

  • Louis DN, Perry A, Reifenberger G et al (2016) The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol (2016) 131:803–820

    Google Scholar 

  • Müller HL (2016) Risk-adapted treatment and follow-up management in childhood-onset craniopharyngioma. Expert Rev Neurother 16(5):535–548

    Article  Google Scholar 

  • Packer RJ, Siegel KR, Sutton N et al (1988) Efficacy of adjuvant chemotherapy for patients with poor-risk medulloblastoma: A preliminary report. Ann Neurol 24:503–511

    Article  CAS  Google Scholar 

  • Packer RJ, Sutton LN, Goldwein JW et al (1991) Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74(3):433–440

    Article  CAS  Google Scholar 

  • Pajtler KW, Witt H, Sill M et al (2015) Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell. 27(5):728–743

    Article  CAS  Google Scholar 

  • Pfister S, Janzarik WG, Remke M et al (2008) BRAF gene duplication constitutes a mechanism of MAPK pathway activation in low-grade astrocytomas. J Clin Invest 118(5):1739–1749

    Article  CAS  Google Scholar 

  • Rutkowski S, Bode U, Deinlein F et al (2005) Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352:978–986

    Article  CAS  Google Scholar 

  • Sturm D, Orr BA, Toprak UH et al (2016) New brain tumor entities emerge from molecular classification of CNS-PNETs. Cell. 164(5):1060–1072

    Article  CAS  Google Scholar 

  • Tait DM, Thorton-Jones BHJG et al (1990) Adjuvant chemotherapy for medulloblastoma: The first multi-centre control trail of the International Society of Pediatric Oncology (SIOP I). Eur J Cancer 26:464–469

    CAS  PubMed  Google Scholar 

  • Taylor MD, Northcott PA, Korshunov A et al (2012) Molecular subgroups of medulloblastoma: the current consensus. Acta Neuropathol 123:465–472

    Article  CAS  Google Scholar 

  • Taylor RE, Bailey CC, Robinson K, Weston CL et al (2003) Results of a randomized study of preradiation chemotherapy versus radiotherapy alone for nonmetastatic medulloblastoma: The International Society of Paediatric Oncology/United Kingdom Children’s Cancer Study Group PNET-3 Study. J Clin Oncol 21:1581–1591

    Article  CAS  Google Scholar 

  • Wu G, Diaz AK, Paugh BS et al (2014) The genomic landscape of diffuse intrinsic pontine glioma and pediatric non-brainstem high-grade glioma. Nat Genet 46(5):444–450

    Article  CAS  Google Scholar 

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Correspondence to Stefan Rutkowski .

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Rutkowski, S., Kortmann, RD. (2021). Hirntumoren. In: Schmoll, HJ. (eds) Kompendium Internistische Onkologie . Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-46764-0_5-1

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  • DOI: https://doi.org/10.1007/978-3-662-46764-0_5-1

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