Additive value of transarterial embolization to systemic sirolimus treatment in kaposiform hemangioendothelioma.

DOI
Publisher DOI
PubMed ID
33231291
Abstract
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 whether additional transarterial embolization is of benefit for children with KHE and KMP. Seventeen 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 grade 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 × 103 /μL, as well as rebound rates of both after cessation of sirolimus were compared. 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. 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.
Date Issued
2021-05-01
Publication Type
Article
Subject(s)
Subjects
Kaposiform hemangioendothelioma Kasabach-Merritt phenomenon coagulopathy embolization
Language(s)
en
Author(s)
Brill, Richard
Uller, Wibke
Huf, Veronika
Müller-Wille, René
Schmid, Irene
Pohl, Alexandra
Häberle, Beate
Perkowski, Sybille
Funke, Katrin
Till, Anne-Marie
Lauten, Melchior
Neumann, Jacob
Güttel, Christian
Heid, Esther
Ziermann, Franziska
Schmid, Axel
Hüsemann, Dieter
Meyer, Lutz
Sporns, Peter B
Schinner, Regina
Schmidt, Vanessa F
Ricke, Jens
Kapp, Friedrich G
Wohlgemuth, Walter A
Wildgruber, Moritz
Additional Credits
Journal
International journal of cancer
Publisher
Wiley-Blackwell
ISSN
0020-7136
Access(Rights)
open.access
Show full item