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Little is known about treatment and outcome of these patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Clinical course, treatment, and outcome in patients with endothelial cell malignancies treated within the Cooperative Weichteilsarkom Studiengruppe (CWS) trials CWS\u201091, \u201096, \u20102002P, and the Soft\u2010Tissue Sarcoma Registry (SoTiSaR) were analyzed (1991\u20102019).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients had angiosarcoma (AS) (<jats:italic>n<\/jats:italic>\u00a0=\u00a012), malignant epithelioid hemangioendothelioma (EHE) (<jats:italic>n<\/jats:italic>\u00a0=\u00a016), and kaposiform hemangioendothelioma (KHE) (<jats:italic>n<\/jats:italic>\u00a0=\u00a013). The median age was 5.39\u00a0years (range, 0.8\u201017.34); 33 patients had localized disease (LD), and 8 patients had metastatic disease. Therapy consisted of chemotherapy (CHT) (AS <jats:italic>n<\/jats:italic>\u00a0=\u00a08, EHE <jats:italic>n<\/jats:italic>\u00a0=\u00a09, KHE <jats:italic>n<\/jats:italic>\u00a0=\u00a05), interferon or new agent therapy (EHE <jats:italic>n<\/jats:italic>\u00a0=\u00a05, 2 KHE <jats:italic>n<\/jats:italic>\u00a0=\u00a02), microscopically or macroscopically complete resection (AS <jats:italic>n<\/jats:italic>\u00a0=\u00a03, EHE <jats:italic>n<\/jats:italic>\u00a0=\u00a06, KHE <jats:italic>n<\/jats:italic>\u00a0=\u00a03), and radiotherapy (AS <jats:italic>n<\/jats:italic>\u00a0=\u00a06, EHE <jats:italic>n<\/jats:italic>\u00a0=\u00a02, KHE <jats:italic>n<\/jats:italic>\u00a0=\u00a01). Two patients (KHE) had watch\u2010and\u2010wait strategy resulting in stable disease. Complete remission (CR) was achieved in AS (10\/12; 83%), EHE (10\/16; 63%), and KHE (5\/13; 38%). The five\u2010year EFS and OS for patients with AS was 64% (\u00b1 29 CI 95%) and 80% (\u00b1 25, CI 95%), with EHE 62% (\u00b1 24, CI 95%) and 78% (\u00b1 23, CI 95%), with KHE 33% (\u00b1 34, CI 95%) and 92% (\u00b1 15, CI 95%), respectively. Complete resection was a significant prognostic factor for AS, LD for EHE.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Endothelial cell malignancies in childhood have a fair outcome with multimodal treatment. New treatment options are needed for metastic disease.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.28095","type":"journal-article","created":{"date-parts":[[2019,12,9]],"date-time":"2019-12-09T03:24:32Z","timestamp":1575861872000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Endothelial cell malignancies in infants, children and adolescents: Treatment results of three Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9551-2399","authenticated-orcid":false,"given":"Monika","family":"Sparber\u2010Sauer","sequence":"first","affiliation":[{"name":"Klinikum Stuttgart, Olgahospital Stuttgart Cancer Center Zentrum f\u00fcr Kinder\u2010 Jugend\u2010 und Frauenmedizin Pediatrics 5 (Oncology, Hematology, Immunology)  Stuttgart 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