Monatsschrift Kinderheilkunde

, 156:1187

Medulloblastome, primitiv neuroektodermale Tumoren und Ependymome

Leitthema

Zusammenfassung

Medulloblastome, primitiv neuroektodermale Tumoren (PNET) und Ependymome sind die häufigsten malignen Hirntumoren des Kindes- und Jugendalters. Seit den 1980er Jahren führten multimodale Therapieoptimierungsstudien der Gesellschaft für Pädiatrische Onkologie und Hämatologie (GPOH) zu verbesserten Überlebensraten. Aktuell werden nach möglichst weitgehender Tumorresektion für die Primärtherapie in der Studie HIT 2000 je nach Histologie, Staging und Alter moderne radio- und/oder chemotherapeutische Konzepte evaluiert. Junge Kinder mit desmoplastischem Medulloblastom erhalten eine alleinige Chemotherapie, bei jungen Kindern mit klassischem Medulloblastom und PNET dagegen soll die Bestrahlung durch Chemotherapie verzögert werden. Bei Metastasierung werden intensivierte Konzepte evaluiert. Beim Ependymom haben Resektion und Lokalbestrahlung große Bedeutung. Für Kinder mit rezidiviertem Medulloblastom, supratentoriellem PNET oder Ependymom werden in der Studie HIT-REZ 2005 eine Lebensverlängerung bei guter Lebensqualität angestrebt sowie neue Medikamente und Therapieformen klinisch geprüft. Für Folgestudien ist ein zentrales Tumorbanking zur verbesserten Therapiestratifizierung mit molekularbiologischen Markern vorgesehen.

Schlüsselwörter

Hirntumoren Medulloblastom PNET Ependymom Therapiestratifizierung  

Medulloblastoma, primitive neuroectodermal tumor, and ependymoma

Abstract

Medulloblastoma, primitive neuroectodermal tumor (PNET), and ependymoma are the most frequent malignant brain tumors of childhood. Since the 1980s, multimodal therapy optimization trials of the German Society of Pediatric Oncology and Hematology (GPOH) have led to improved survival rates. Within the current primary treatment trial HIT 2000, modern strategies for radiotherapy and chemotherapy are evaluated according to histology, staging, and age after maximal safe tumor resection. Young children with desmoplastic medulloblastoma receive chemotherapy alone, and the aim for young children with classic medulloblastoma and PNET is for chemotherapy to delay radiotherapy. Intensified treatment concepts are evaluated in metastatic disease. In ependymoma, the extent of resection and local radiotherapy are most important. Prolonging survival while preserving a good quality of life is the aim of the trial HIT-REZ 2005 for children with relapsed medulloblastoma, PNET, or ependymoma; it includes the evaluation of new drugs and treatment modalities (temozolomide, intraventricular etoposide). Central tumor banking and improved treatment stratification by prognostic molecular markers are planned for future trials.

Keywords

Brain tumors Medulloblastoma PNET Ependymoma Treatment stratification 

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Copyright information

© Springer Medizin Verlag 2008

Authors and Affiliations

  1. 1.Kinderklinik und Poliklinik Universitätsklinikum WürzburgWürzburgDeutschland
  2. 2.Abteilung Pädiatrische Hämatologie/OnkologieZentrum für Kinderheilkunde, Universitätsklinikum BonnBonnDeutschland

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