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Endothelial cell ma...
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Sparber-Sauer, MonikaKlinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany
(författare)
Endothelial cell malignancies in infants, children and adolescents : Treatment results of three Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
- Artikel/kapitelEngelska2020
Förlag, utgivningsår, omfång ...
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2019-12-08
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WILEY,2020
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printrdacarrier
Nummerbeteckningar
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LIBRIS-ID:oai:DiVA.org:uu-408222
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https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-408222URI
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https://doi.org/10.1002/pbc.28095DOI
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Språk:engelska
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Sammanfattning på:engelska
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Ämneskategori:ref swepub-contenttype
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Ämneskategori:art swepub-publicationtype
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Background Endothelial cell malignancies are extremely rare in childhood. New identification of genetic abnormalities (WWTR1:CAMTA1 translocation) helps to recognize potential therapeutic targets. Little is known about treatment and outcome of these patients. Methods Clinical course, treatment, and outcome in patients with endothelial cell malignancies treated within the Cooperative Weichteilsarkom Studiengruppe (CWS) trials CWS-91, -96, -2002P, and the Soft-Tissue Sarcoma Registry (SoTiSaR) were analyzed (1991-2019). Results Patients had angiosarcoma (AS) (n = 12), malignant epithelioid hemangioendothelioma (EHE) (n = 16), and kaposiform hemangioendothelioma (KHE) (n = 13). The median age was 5.39 years (range, 0.8-17.34); 33 patients had localized disease (LD), and 8 patients had metastatic disease. Therapy consisted of chemotherapy (CHT) (AS n = 8, EHE n = 9, KHE n = 5), interferon or new agent therapy (EHE n = 5, 2 KHE n = 2), microscopically or macroscopically complete resection (AS n = 3, EHE n = 6, KHE n = 3), and radiotherapy (AS n = 6, EHE n = 2, KHE n = 1). Two patients (KHE) had watch-and-wait 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-year EFS and OS for patients with AS was 64% (+/- 29 CI 95%) and 80% (+/- 25, CI 95%), with EHE 62% (+/- 24, CI 95%) and 78% (+/- 23, CI 95%), with KHE 33% (+/- 34, CI 95%) and 92% (+/- 15, CI 95%), respectively. Complete resection was a significant prognostic factor for AS, LD for EHE. Conclusions Endothelial cell malignancies in childhood have a fair outcome with multimodal treatment. New treatment options are needed for metastic disease.
Ämnesord och genrebeteckningar
Biuppslag (personer, institutioner, konferenser, titlar ...)
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Koscielniak, EwaKlinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany;Childrens Hosp, Dept Pediat Hematol & Oncol, Tubingen, Germany
(författare)
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Vokuhl, ChristianKiel Pediat Tumor Registry, Sect Pediat Pathol, Dept Pathol, Kiel, Germany
(författare)
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Schmid, IreneLudwig Maximilians Univ Munchen, Dr Hauner Childrens Hosp, Div Pediat Hematol & Oncol, Munich, Germany
(författare)
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Bien, EwaMed Univ Gdansk, Dept Pediat Hematol Oncol & Endocrinol, Gdansk, Poland
(författare)
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Seitz, GuidoUniv Childrens Hosp Marburg, Dept Pediat Surg, Marburg, Germany
(författare)
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Hallmen, ErikaKlinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany
(författare)
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von Kalle, TheklaKlinikum Stuttgart, Inst Radiol, Stuttgart Canc Ctr,Zentrum Kinder Jugend & Frauen, Olgahosp, Stuttgart, Germany
(författare)
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Scheer, MonikaKlinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany
(författare)
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Muenter, MarcKlinikum Stuttgart, Inst Radiotherapy, Stuttgart, Germany
(författare)
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Bielack, Stefan S.Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany;Univ Munster, Dept Pediat Hematol & Oncol, Munster, Germany
(författare)
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Niggli, FelixUniv Zurich, Dept Pediat Oncol, Zurich, Switzerland
(författare)
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Ljungman, Gustaf,1958-Uppsala universitet,Barnneurologi/Barnonkologi,Childrens Univ Hosp, Uppsala, Sweden(Swepub:uu)gustaflm
(författare)
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Fuchs, JoergUniv Childrens Hosp, Dept Pediat Surg & Urol, Tubingen, Germany
(författare)
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Hettmer, SimoneUniv Freiburg, Div Pediat Hematol & Oncol, Dept Pediat & Adolescent Med, Fac Med, Freiburg, Germany
(författare)
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Roessler, JochenUniv Bern, Univ Klin Kinderheilkunde Hamatol & Onkol, Inselspital, Bern, Switzerland
(författare)
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Klingebiel, ThomasGoethe Univ Frankfurt, Dept Children & Adolescents, Frankfurt, Germany
(författare)
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Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, GermanyKlinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany;Childrens Hosp, Dept Pediat Hematol & Oncol, Tubingen, Germany
(creator_code:org_t)
Sammanhörande titlar
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Ingår i:Pediatric Blood & Cancer: WILEY67:31545-50091545-5017
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Sparber-Sauer, M ...
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Koscielniak, Ewa
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Vokuhl, Christia ...
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Schmid, Irene
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Bien, Ewa
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Seitz, Guido
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visa fler...
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Hallmen, Erika
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von Kalle, Thekl ...
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Scheer, Monika
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Muenter, Marc
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Bielack, Stefan ...
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Niggli, Felix
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Ljungman, Gustaf ...
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Fuchs, Joerg
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Hettmer, Simone
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Roessler, Jochen
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Klingebiel, Thom ...
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