Additive value of transarterial embolization to systemic sirolimus treatment in kaposiform hemangioendothelioma
| Autor: | Lutz Meyer, Richard Brill, Wibke Uller, Regina Schinner, Franziska Ziermann, Vanessa Franziska Schmidt, Jochen Rössler, Peter B. Sporns, Katrin Funke, Irene Schmid, Christian Güttel, Melchior Lauten, Beate Häberle, Esther Heid, Moritz Wildgruber, Axel Schmid, Dieter Hüsemann, Anne-Marie Till, Sybille Perkowski, Alexandra Pohl, Veronika Huf, Jens Ricke, René Müller-Wille, Walter A. Wohlgemuth, Jacob Neumann, Friedrich G. Kapp |
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| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
615 Pharmacology & therapeutics
prescription drugs Male Cancer Research medicine.medical_specialty medicine.medical_treatment 610 Medicine & health Kasabach-Merritt Syndrome Targeted therapy 03 medical and health sciences 0302 clinical medicine Transarterial embolization Coagulopathy medicine Humans Embolization ddc:610 Sarcoma Kaposi Retrospective Studies Sirolimus medicine.diagnostic_test business.industry Magnetic resonance imaging medicine.disease equipment and supplies Embolization Therapeutic Primary tumor 3. Good health Surgery ddc Oncology Kaposiform Hemangioendothelioma 030220 oncology & carcinogenesis Hemangioendothelioma Vascular tumor Female business medicine.drug |
| Zdroj: | 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 |
| Popis: | Purpose: 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 if additional transarterial embolization is of benefit for children with KHE and KMP. Methods: 17 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 rate 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 103/µl, as well as rebound rates of both after cessation of sirolimus were compared. Results: 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. Conclusion: 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. |
| Databáze: | OpenAIRE |
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