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Perioperative Intensity-Modulated Brachytherapy for Refractory Orbital Rhabdomyosarcomas in Children

Perioperative intensitätsmodulierte Brachytherapie bei Kindern mit refraktären orbitalen Rhabdomyosarkomen

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Purpose:

To evaluate the feasibility and toxicity of perioperative intensity-modulated brachytherapy (IMBT) as well as functional outcome in children with therapy-refractory orbital rhabdomyosarcomas (RMS).

Patients and Methods:

Since 1993, children with therapy-refractory orbital RMS have been treated by a multidisciplinary approach combining function-preserving, mostly R1 tumor resection and perioperative IMBT at the University Hospital of Schleswig-Holstein, Germany. All children with orbital RMS, who were enrolled in this multidisciplinary treatment protocol between 1993 and 2002, were prospectively assessed with respect to evaluation of side effects and functional outcome.

Results:

Ten children (six boys, four girls) were included. Median age was 6.5 years (range, 1–19 years) at the beginning of our treatment and 6.0 years (range 1–17 years) at diagnosis. All children were in Intergroup Rhabdomyosarcoma Study Group (IRSG) group III and had embryonal subtype. Estimated 5-year survival was 62% ± 18%. There was no radiation-related toxicity grade 3 or 4 observed. The eyes were primarily preserved in all cases. One child underwent secondary orbital exenteration 10 months after completion of IMBT. Visual acuity could be preserved apart from one child developing significant visual deterioration due to radiation cataract grade 2. The cosmetic results were good or very good in eight and moderate in two children. Four children died of their disease.

Conclusion:

This interdisciplinary, individually tailored and function-preserving treatment procedure has proven to be a well-tolerated therapeutic option in cases with refractory orbital RMS. It provides both improvement of local tumor control and quality of life.

Ziel:

Die Durchführbarkeit und Verträglichkeit der perioperativen intensitätsmodulierten Brachytherapie (IMBT) bei Kindern mit refraktären orbitalen Rhabdomyosarkomen (RMS) sowie deren funktionelles Ergebnis sollten untersucht werden.

Patienten und Methodik:

Seit 1993 werden Kinder mit therapierefraktären orbitalen RMS mittels einer multidisziplinären Behandlungsmethode am Universitätsklinikum Schleswig-Holstein behandelt, welche eine funktionserhaltende, meist R1-Tumorresektion mit einer perioperativen IMBT kombiniert. Ausgewertet wurden alle Kinder mit orbitalem RMS, die zwischen 1993 und 2002 in dieses multidisziplinäre Behandlungsprotokoll eingeschlossen und prospektiv hinsichtlich Verträglichkeit und funktionellem Ergebnis untersucht worden waren.

Ergebnisse:

Es wurden zehn Kinder (sechs Jungen, vier Mädchen) behandelt. Das mediane Alter betrug 6,5 Jahre (Spannweite 1–19 Jahre) bei unserem Behandlungsbeginn und 6,0 Jahre (Spannweite 1–17 Jahre) bei Diagnosestellung. Bei allen Kindern lagen ein Stadium III gemäß Intergroup Rhabdomyosarcoma Study Group (IRSG) und ein embryonaler Subtyp vor. Die geschätzte 5-Jahres-Überlebensrate betrug 62% ± 18%. Es wurde keine Strahlentoxizität Grad 3 oder 4 beobachtet. Die Augen wurden primär in allen Fällen erhalten, nur bei einem Kind war 10 Monate nach IMBT eine sekundäre Orbitaexenteration erforderlich. Die Sehschärfe wurde überwiegend geschont. Lediglich ein Kind entwickelte eine signifikante Sehverschlechterung aufgrund einer radiogenen Katarakt Grad 2. Die kosmetischen Ergebnisse waren bei acht Kindern gut oder sehr gut und bei zwei Kindern moderat. Vier Kinder starben an ihrem Tumor.

Schlussfolgerung:

Diese interdisziplinäre, individuell zugeschnittene funktionserhaltende Behandlungsprozedur hat sich als gut verträgliche palliative therapeutische Option in Fällen refraktärer orbitaler RMS erwiesen. Sie ermöglicht sowohl eine Verbesserung der lokalen Tumorkontrolle als auch der Lebensqualität.

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Author information

Authors and Affiliations

  1. Department of Neurosurgery, University Clinic Schleswig-Holstein, Campus Kiel, Germany

    Rainer Joachim Strege & Maximilian H. Mehdorn

  2. Interdisciplinary Brachytherapy Unit, University Clinic Schleswig-Holstein, Campus Lübeck, Germany

    György Kovács MD, PhD

  3. Department of Head-and-Neck Surgery, University Clinic Schleswig-Holstein, Campus Lübeck, Germany

    Jens Eduard Meyer

  4. Department of Ophthalmology, University Clinic Schleswig-Holstein, Campus Kiel, Germany

    Detlef Holland

  5. Department of Pediatrics, University Clinic Schleswig-Holstein, Campus Kiel, Germany

    Alexander Claviez

  6. Professor of Radiotherapy & Head, Interdisciplinary Brachytherapy Unit, University Clinic Schleswig-Holstein, Ratzeburger Allee 160, 23538, Lübeck, Germany

    György Kovács MD, PhD

Authors
  1. Rainer Joachim Strege
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  2. György Kovács MD, PhD
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  3. Jens Eduard Meyer
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  4. Detlef Holland
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  5. Alexander Claviez
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  6. Maximilian H. Mehdorn
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Corresponding author

Correspondence to György Kovács MD, PhD.

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*All authors contributed equally to the study.

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Strege, R.J., Kovács, G., Meyer, J.E. et al. Perioperative Intensity-Modulated Brachytherapy for Refractory Orbital Rhabdomyosarcomas in Children. Strahlenther Onkol 185, 789–798 (2009). https://doi.org/10.1007/s00066-009-2012-x

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  • DOI: https://doi.org/10.1007/s00066-009-2012-x

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