Brill, Richard and Uller, Wibke and Huf, Veronika and Mueller-Wille, Rene and Schmid, Irene and Pohl, Alexandra and Haeberle, Beate and Perkowski, Sybille and Funke, Katrin and Till, Anne-Marie and Lauten, Melchior and Neumann, Jacob and Guettel, Christian and Heid, Esther and Ziermann, Franziska and Schmid, Axel and Huesemann, Dieter and Meyer, Lutz and Sporns, Peter B. and Schinner, Regina and Schmidt, Vanessa F. and Ricke, Jens and Roessler, Jochen and Kapp, Friedrich G. and Wohlgemuth, Walter A. and Wildgruber, Moritz (2021) Additive value of transarterial embolization to systemic sirolimus treatment in kaposiform hemangioendothelioma. INTERNATIONAL JOURNAL OF CANCER, 148 (9). pp. 2345-2351. ISSN 0020-7136, 1097-0215
Full text not available from this repository. (Request a copy)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 x 10(3)/mu L, 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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | KASABACH-MERRITT PHENOMENON; RISKS; coagulopathy; embolization; Kaposiform hemangioendothelioma; Kasabach‐ Merritt phenomenon |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Röntgendiagnostik |
| Depositing User: | Petra Gürster |
| Date Deposited: | 21 Apr 2021 14:03 |
| Last Modified: | 21 Apr 2021 14:03 |
| URI: | https://pred.uni-regensburg.de/id/eprint/43179 |
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