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Additive value of Transarterial Embolization to systemic Sirolimus treatment in Kaposiform Hemangioendothelioma
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  • Richard Brill,
  • Wibke Uller,
  • Veronika Huf,
  • René Müller-Wille,
  • Irene Schmid,
  • Alexandra Pohl,
  • Beate Haeberle,
  • Sybille Perkowski,
  • Katrin Funke,
  • Anne-Marie Till,
  • Melchior Lauten,
  • Jacob Neumann,
  • Chrsitian Güttel,
  • Esther Heid,
  • Franziska Ziermann,
  • Axel Schmid,
  • Dieter Hüsemann,
  • Lutz Mayr,
  • Peter Sporns,
  • Regina Schinner,
  • Vanessa Schmidt,
  • Jens Ricke,
  • Jochen Rössler,
  • Friedrich Kapp,
  • Walter Wohlgemuth,
  • Moritz Wildgruber
Richard Brill
Universität Halle-Wittenberg
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Wibke Uller
Universität Regensburg
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Veronika Huf
Universität Regensburg
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René Müller-Wille
Universität Göttingen Medizinische Fakultät
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Irene Schmid
Children`s Hospital of the Ludwig-Maximilians-University, Munich
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Alexandra Pohl
Ludwig-Maximilians-Universitat Munchen
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Beate Haeberle
University of Munich
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Sybille Perkowski
Universität Münster
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Katrin Funke
Universität Münster
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Anne-Marie Till
Universität zu Lübeck
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Melchior Lauten
University Hospital Schleswig-Holstein
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Jacob Neumann
HELIOS Kliniken GmbH
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Chrsitian Güttel
HELIOS Kliniken GmbH
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Esther Heid
Technische Universitat Munchen
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Franziska Ziermann
Technische Universitat Munchen
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Axel Schmid
Friedrich-Alexander-Universität Erlangen-Nürnberg
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Dieter Hüsemann
GLG Werner Forssmann Klinikum Eberswalde
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Lutz Mayr
GLG Werner Forssmann Klinikum Eberswalde
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Peter Sporns
Universität Basel
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Regina Schinner
Ludwig-Maximilians-Universitat Munchen
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Vanessa Schmidt
Ludwig-Maximilians-Universitat Munchen
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Jens Ricke
Ludwig-Maximilians-Universitat Munchen
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Jochen Rössler
Universitatsklinikum Freiburg
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Friedrich Kapp
University Medical Center Freiburg
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Walter Wohlgemuth
Universität Halle-Wittenberg
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Moritz Wildgruber
Ludwig-Maximilians-Universitat Munchen
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Abstract

Purpose: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor in children, which can be accompanied by life-threatening thrombocytopenia, referred to as Kasabach-Merritt Phenomenon (KMP). The mTOR inhibitor sirolimus is emerging as targeted therapy in KHE. As the sirolimus effect on KHE occurs only after several weeks we aimed to evaluate if additional transarterial embolization is of benefit for children with KHE and KMP. Methods: 17 patients with KHE and KMP acquired from 11 hospitals in Germany were retrospectively divided into two cohorts. Children being treated with adjunct transarterial embolization and systemic sirolimus, and those being treated with sirolimus without additional embolization. Bleeding rate as defined by WHO was determined for all patients. Response of the primary tumor at 6 and 12 months assessed by Magnetic Resonance Imaging (MRI), time to response of KMP defined as thrombocyte increase >150 x 103/µl, as well as rebound rates of both after cessation of sirolimus were compared. Results: N= 8 patients had undergone additive embolization to systemic sirolimus therapy, sirolimus in this group was started after a mean of 6.5 ± 3 days following embolization. N=9 patients were identified who had received sirolimus without additional embolization. Adjunct embolization induced a more rapid resolution of KMP within a median of 7 days vs 3 months, however tumor response as well as rebound rates were similar between both groups. Conclusion: Additive embolization may be of value for a more rapid rescue of consumptive coagulopathy in children with KHE and KMP compared to systemic sirolimus only.