Successful management of Kaposiform Hemangioendothelioma with long-term sirolimus treatment: a case report and review of the literature

Matteo Chinello, Daniela Di Carlo, Francesca Olivieri, Rita Balter, Massimiliano De Bortoli, Virginia Vitale, Ada Zaccaron, Elisa Bonetti, Alice Parisi, Simone Cesaro
  • Daniela Di Carlo
    Mother and Child Department, University of Verona, Italy
  • Francesca Olivieri
    Mother and Child Department, University of Verona,
  • Rita Balter
    Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy, Italy
  • Massimiliano De Bortoli
    Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy, Italy
  • Virginia Vitale
    Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy, Italy
  • Ada Zaccaron
    Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy, Italy
  • Elisa Bonetti
    Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy, Italy
  • Alice Parisi
    Department of Pathological Anatomy, Azienda Ospedaliera Universitaria Integrata, Verona, Italy., Italy
  • Simone Cesaro
    Pediatric Hematology Oncology, Azienda Ospedaliera Universitaria Integrata, Verona, Italy, Italy

Abstract

Background: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumour of the infancy and of the first decade of life. It is locally aggressive and potentially life threatening when associated to consumptive coagulopathy, known as Kasabach-Merritt syndrome (KMS). No consensus or guideline for the therapy has been reached because of the lack of prospective trials and the different standard care suggestions are based on retrospective case series.

Case report: We report the case of a 9-month-old male with KHE and KMS in which the initial response, obtained with prednisone and vincristine, was subsequently consolidated and strengthened by long-term treatment with sirolimus, an mTOR inhibitor. A summary of the published data is presented as well.

Conclusions: The inhibition of mTOR pathway represents the most important therapeutic innovation introduced in the last few years for KHE. Our case shows the effectiveness and good tolerance of long-term therapy with sirolimus.

Keywords: Kaposiform Hemangioendothelioma, Kasabach-Merrit syndrome, sirolimus, prednisone, vincristine

Keywords

Kaposiform Hemangioendothelioma, Kasabach-Merrit syndrome, sirolimus, prednisone, vincristine

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Submitted: 2018-04-25 19:50:41
Published: 2018-07-01 00:00:00
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