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Endothelial cell ma...
Endothelial cell malignancies in infants, children and adolescents : Treatment results of three Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry
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- Sparber-Sauer, Monika (author)
- Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany
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- Koscielniak, Ewa (author)
- Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany;Childrens Hosp, Dept Pediat Hematol & Oncol, Tubingen, Germany
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- Vokuhl, Christian (author)
- Kiel Pediat Tumor Registry, Sect Pediat Pathol, Dept Pathol, Kiel, Germany
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- Schmid, Irene (author)
- Ludwig Maximilians Univ Munchen, Dr Hauner Childrens Hosp, Div Pediat Hematol & Oncol, Munich, Germany
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- Bien, Ewa (author)
- Med Univ Gdansk, Dept Pediat Hematol Oncol & Endocrinol, Gdansk, Poland
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- Seitz, Guido (author)
- Univ Childrens Hosp Marburg, Dept Pediat Surg, Marburg, Germany
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- Hallmen, Erika (author)
- Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany
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- von Kalle, Thekla (author)
- Klinikum Stuttgart, Inst Radiol, Stuttgart Canc Ctr,Zentrum Kinder Jugend & Frauen, Olgahosp, Stuttgart, Germany
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- Scheer, Monika (author)
- Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany
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- Muenter, Marc (author)
- Klinikum Stuttgart, Inst Radiotherapy, Stuttgart, Germany
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- Bielack, Stefan S. (author)
- Klinikum Stuttgart, Olgahosp, Stuttgart Canc Ctr, Zentrum Kinder Jugend & Frauenmed,Pediat Oncol He, Stuttgart, Germany;Univ Munster, Dept Pediat Hematol & Oncol, Munster, Germany
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- Niggli, Felix (author)
- Univ Zurich, Dept Pediat Oncol, Zurich, Switzerland
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- Ljungman, Gustaf, 1958- (author)
- Uppsala universitet,Barnneurologi/Barnonkologi,Childrens Univ Hosp, Uppsala, Sweden
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- Fuchs, Joerg (author)
- Univ Childrens Hosp, Dept Pediat Surg & Urol, Tubingen, Germany
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- Hettmer, Simone (author)
- Univ Freiburg, Div Pediat Hematol & Oncol, Dept Pediat & Adolescent Med, Fac Med, Freiburg, Germany
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- Roessler, Jochen (author)
- Univ Bern, Univ Klin Kinderheilkunde Hamatol & Onkol, Inselspital, Bern, Switzerland
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- Klingebiel, Thomas (author)
- Goethe Univ Frankfurt, Dept Children & Adolescents, Frankfurt, Germany
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(creator_code:org_t)
- 2019-12-08
- 2020
- English.
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In: Pediatric Blood & Cancer. - : WILEY. - 1545-5009 .- 1545-5017. ; 67:3
- Related links:
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https://urn.kb.se/re...
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https://doi.org/10.1...
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Abstract
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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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Keyword
- angiosarcoma
- children
- CWS Group
- endothelial cell malignancies
- epithelioid hemangioendothelioma
- kaposiform hemangioendothelioma
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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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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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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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
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Pediatric Blood ...
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Uppsala University