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

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; ... (2021). Additive value of transarterial embolization to systemic sirolimus treatment in kaposiform hemangioendothelioma. International journal of cancer, 148(9), pp. 2345-2351. Wiley-Blackwell 10.1002/ijc.33406

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Haematology/Oncology

UniBE Contributor:

Rössler, Jochen Karl

Subjects:

600 Technology > 610 Medicine & health
600 Technology > 610 Medicine & health > 615 Pharmacology & therapeutics, prescription drugs

ISSN:

0020-7136

Publisher:

Wiley-Blackwell

Language:

English

Submitter:

Jochen Karl Rössler

Date Deposited:

18 Jan 2021 11:55

Last Modified:

18 Mar 2021 01:33

Publisher DOI:

10.1002/ijc.33406

PubMed ID:

33231291

Uncontrolled Keywords:

Kaposiform hemangioendothelioma Kasabach-Merritt phenomenon coagulopathy embolization

BORIS DOI:

10.48350/150726

URI:

https://boris.unibe.ch/id/eprint/150726

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