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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

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

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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.

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.

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Authors and Affiliations

  1. Department of Radiotherapy and Radiooncology, University of Leipzig, Leipzig, Germany

    Klaus Müller

  2. Department of Radiotherapy and Radiooncology, Katharinenhospital, Stuttgart, Germany

    Helmut Welker

  3. Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart – Olgahospital, Stuttgart, Germany

    Eberhard Maaß

  4. Department of Radiotherapy and Radiooncology, University of Cologne, Cologne, Germany

    Rudolf Bongartz

  5. Department of Pediatric Oncology, University of Cologne, Cologne, Germany

    Frank Berthold

  6. Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

    André von Bueren & Stefan Rutkowski

  7. Department of Neuropathology, University of Bonn Medical Center, Bonn, Germany

    Torsten Pietsch

  8. Department of Neuroradiology, University of Wuerzburg, Wuerzburg, Germany

    Monika Warmuth-Metz

  9. Institute for Medical Biostatistics, Epidemiology and Informatics, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany

    Isabella Zwiener

  10. Department of Radiotherapy and Radiooncology, University of Leipzig, Stephanstr. 9a, 04103, Leipzig, Germany

    Klaus Müller

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  1. Klaus Müller
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Correspondence to Klaus Müller.

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Müller, K., Zwiener, I., Welker, H. et al. Curative treatment for central nervous system medulloepithelioma despite residual disease after resection. Strahlenther Onkol 187, 757–762 (2011). https://doi.org/10.1007/s00066-011-2256-0

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