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Strahlentherapie und Onkologie

, Volume 187, Issue 11, pp 757–762 | Cite as

Curative treatment for central nervous system medulloepithelioma despite residual disease after resection

Report of two cases treated according to the GPHO protocol HIT 2000 and review of the literature
  • Klaus Müller
  • Isabella Zwiener
  • Helmut Welker
  • Eberhard Maaß
  • Rudolf Bongartz
  • Frank Berthold
  • Torsten Pietsch
  • Monika Warmuth-Metz
  • André von Bueren
  • Stefan Rutkowski
Case Study

Abstract

Medulloepithelioma of the central nervous system (CNS) is an uncommon primitive neuroectodermal tumor (PNET) usually occurring in early childhood. It is characterized by highly malignant behavior with a propensity for progression, recurrence, and dissemination despite intensive therapy. Due to its rarity, the optimal management is still unknown. However, gross total resection (GTR) has been considered crucial to achieve cure.

In this article, the authors report on 2 cases of CNS medulloepithelioma in which long-term survival (more than 6 years) could be achieved despite evidence of, or suspected postoperative residual disease with an otherwise dismal prognosis.

The patients were treated according to different strata of the protocol for primitive neuroectodermal tumors (PNET) of the German–Austrian multicenter trial of the German Society for Pediatric Oncology and Hematology (GPOH) for childhood brain tumors (HIT 2000). Treatment included postoperative hyperfractionated radiotherapy of the craniospinal axis followed by a boost to the tumor site in combination with chemotherapy.

A review of the 2 reported and 37 previously published cases confirmed GTR and older age as positive prognostic factors.

Key Words

Medulloepithelioma Long-term survival Hyperfractionated radiotherapy Residual disease HIT 2000 Chemotherapy 

Kurative Behandlung von inkomplett resezierten Medulloepitheliomen des ZNS. Bericht über zwei Fälle, die nach dem GPHO-Protokoll HIT 2000 behandelt wurden, und Literaturdurchsicht

Zusammenfassung

Das Medulloepitheliom des zentralen Nervensystems ist ein seltener, primitiver, neuroektodermaler Tumor, der gewöhnlich in der frühen Kindheit auftritt. Es ist durch ein äußerst bösartiges Verhalten gekennzeichnet und neigt auch nach intensiver Therapie zu Progression, Rezidivbildung und Metastasierung. Aufgrund seiner Seltenheit ist die optimale Behandlung unbekannt. Dennoch ging man bisher davon aus, dass eine Totalresektion für einen kurativen Therapieansatz unabdingbar sei.

Wir berichten nun über zwei Fälle von ZNS-Medulloepitheliomen, bei denen ein Langzeitüberleben (länger als 6 Jahre) erreicht werden konnte, obwohl mit Sicherheit beziehungsweise höchstwahrscheinlich postoperativ Resttumorgewebe in situ verblieben war.

Die Patienten wurden entsprechend verschiedenen Zweigen des Protokolls für primitive, neuroektodermale Tumore einer deutsch-österreichischen, multizentrischen Studie für kindliche Hirntumore (HIT 2000) der Deutschen Gesellschaft für Pädiatrische Hämatologie und Onkologie (GPOH) behandelt. Die Therapien umfassten eine postoperative, hyperfraktionierte Bestrahlung der kraniospinalen Achse mit Boostbestrahlung der Tumorregion sowie eine begleitende Chemotherapie.

Eine Auswertung der beiden vorgestellten und 37 weiterer, bereits publizierter Fälle bestätigte eine Totalresektion (Figure 5) und ein höheres Alter (Figure 3) als positive Prognosefaktoren.

Schlüsselwörter

Medulloepitheliom Langzeitüberleben Hyperfraktionierte Bestrahlung Inkomplette Resektion HIT 2000 Chemotherapie 

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Copyright information

© Urban & Vogel 2011

Authors and Affiliations

  • Klaus Müller
    • 1
    • 10
  • Isabella Zwiener
    • 9
  • Helmut Welker
    • 2
  • Eberhard Maaß
    • 3
  • Rudolf Bongartz
    • 4
  • Frank Berthold
    • 5
  • Torsten Pietsch
    • 7
  • Monika Warmuth-Metz
    • 8
  • André von Bueren
    • 6
  • Stefan Rutkowski
    • 6
  1. 1.Department of Radiotherapy and RadiooncologyUniversity of LeipzigLeipzigGermany
  2. 2.Department of Radiotherapy and RadiooncologyKatharinenhospitalStuttgartGermany
  3. 3.Pediatrics 5 (Oncology, Hematology, Immunology)Klinikum Stuttgart – OlgahospitalStuttgartGermany
  4. 4.Department of Radiotherapy and RadiooncologyUniversity of CologneCologneGermany
  5. 5.Department of Pediatric OncologyUniversity of CologneCologneGermany
  6. 6.Department of Pediatric Hematology and OncologyUniversity Medical Center Hamburg-EppendorfHamburgGermany
  7. 7.Department of NeuropathologyUniversity of Bonn Medical CenterBonnGermany
  8. 8.Department of NeuroradiologyUniversity of WuerzburgWuerzburgGermany
  9. 9.Institute for Medical Biostatistics, Epidemiology and InformaticsUniversity Medical Center of the Johannes Gutenberg University MainzMainzGermany
  10. 10.Department of Radiotherapy and RadiooncologyUniversity of LeipzigLeipzigGermany

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