| Summary: | [english] We report on a case of Klippel-Trenaunay syndrome with a histologically diagnosed dedifferentiated angiosarcoma. During operative tumor debulking, a disseminated bleeding developed which required a massive transfusion of red blood cell concentrates and therapeutic plasma. While plasmatic coagulation was within normal range, thromboelastometry showed a distinct hyperfibrinolysis. The bleeding was controlled by prolonged cauterisation and the substitution of tranexam acid as well as fresh frozen plasma. As a result of this treatment, hyperfibrinolysis showed complete restitution within a few hours. Histologically, a malignant peripheral nerve sheat tumor, a dedifferentiated angiosarcoma, a spindle cell sarcoma and an osteosarcoma were diagnosed within the tissue samples.
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