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Decreased vascularization of retroperitoneal kaposiform hemangioendothelioma induced by treatment with sirolimus explains relief of symptoms

  • Ionela Iacobas
    Correspondence
    Corresponding author. Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, 1102 Bates Street, suite 1025.06, Houston, TX 77030. Tel.: +1-832-824-4819; fax: +1-832-825-4846.
    Affiliations
    Drexel University College of Medicine, Pediatric Hematology-Oncology, Saint Peter's University Hospital, 254 Easton Avenue, New Brunswick, NJ 08901
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  • Author Footnotes
    1 Tel.: +1-732-235-7721.
    Mitchell L. Simon
    Footnotes
    1 Tel.: +1-732-235-7721.
    Affiliations
    UMDNJ-Robert Wood Johnson Medical School, Department of Radiology, MEB #404, P.O. Box 19, New Brunswick, NJ 08903–0019
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  • Tali Amir
    Affiliations
    UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ 08903–0019
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  • Author Footnotes
    1 Tel.: +1-732-235-7721.
    Christopher E. Gribbin
    Footnotes
    1 Tel.: +1-732-235-7721.
    Affiliations
    UMDNJ-Robert Wood Johnson Medical School, Department of Radiology, MEB #404, P.O. Box 19, New Brunswick, NJ 08903–0019
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  • Author Footnotes
    2 Tel.: +1-732-390-1160; fax: +1-732-390-8449.
    Thomas G. McPartland
    Footnotes
    2 Tel.: +1-732-390-1160; fax: +1-732-390-8449.
    Affiliations
    Saint Peter's University Hospital, Pediatric Orthopedic Surgery, 254 Easton Avenue, New Brunswick, NJ 08901
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  • Author Footnotes
    3 Tel.: +1-732-613-2929; fax: +1-732-747-2606.
    Matthew R. Kaufman
    Footnotes
    3 Tel.: +1-732-613-2929; fax: +1-732-747-2606.
    Affiliations
    Drexel University College of Medicine, Saint Peter's University Hospital, Department of Plastic Surgery, 254 Easton Avenue, New Brunswick, NJ 08901
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  • Author Footnotes
    4 Tel.: +1-732-745-6674; fax: +1-732-418-9708.
    Stanley Calderwood
    Footnotes
    4 Tel.: +1-732-745-6674; fax: +1-732-418-9708.
    Affiliations
    Drexel University College of Medicine, Pediatric Hematology-Oncology, Saint Peter's University Hospital, 254 Easton Avenue, New Brunswick, NJ 08901
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  • Author Footnotes
    1 Tel.: +1-732-235-7721.
    John L. Nosher
    Footnotes
    1 Tel.: +1-732-235-7721.
    Affiliations
    UMDNJ-Robert Wood Johnson Medical School, Department of Radiology, MEB #404, P.O. Box 19, New Brunswick, NJ 08903–0019
    Search for articles by this author
  • Author Footnotes
    1 Tel.: +1-732-235-7721.
    2 Tel.: +1-732-390-1160; fax: +1-732-390-8449.
    3 Tel.: +1-732-613-2929; fax: +1-732-747-2606.
    4 Tel.: +1-732-745-6674; fax: +1-732-418-9708.

      Abstract

      Kaposiform hemangioendothelioma (KHE) is a vascular tumor with poor prognosis. We present a child with progressive disability, extreme pain, and autonomic dysfunction due to a retroperitoneal KHE where radiologic characteristics were essential for diagnosis and monitoring of response to therapy. He received sirolimus, and the symptomatology resolved completely. Repeat MRIs revealed fast marked decrease in vascularity of the tumor, although the volume was not significantly affected. We suggest that the sirolimus-induced tumor de-vascularization may explain the clinical and coagulopathy improvement.

      Keywords

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